thalassemia study in indiashodhganga.inflibnet.ac.in/bitstream/10603/77029/11/12_chapter-3.pdf ·...

Survey of blood transfusion-induced malaria and other diseases in Thalassemia patients from Solapur District (M.S.) India.

47

Gorgan, Iran. Screening for Thalassemia and Hemoglobinopathies in Canada observed by

Bijayini et al. (2010). The observations and study on BT was studied by Cao and Galanello

(2010). James et al., (2010) elevated exhaled carbon monoxide concentration in

hemoglobinopathies and its relation to red blood cell transfusion therapy. Hira et al. (2011).

Find out the complications in thalassemia patients receiving blood transfusion. Thompson et

al., (2011) investigated the red cell alloimmunization in a diverse population of transfused

patients with thalassemia. Recently, Abdelmohsen (2011) studied the exhaled carbon

monoxide concentration in beta-thalassemia and its relation to red blood cell transfusion

therapy in pediatrics. Cappellini et al., (2011) observed iron chelation with deferasirox in

adult and pediatric patients with thalassemia major. Recently Arıca et al. (2012) evaluate the

hemoglobinopathy screening results of a six year period in Turkey. Fabrice et al (2012),

observed the genetic modifiers of beta-thalassemia and clinical severity as assessed by age at

first transfusion. Marion et al. (2012) studied the 0-thalassemia deletion in a Greek patient

with HbH disease and β-thalassemia trait. Yixuan et al. (2012) studied genetic correction of

β-thalassemia patient-specific iPS cells and its use in improving hemoglobin production in

irradiated SCID mice.

Thalassemia study in India

Sood et al. (1993) in his report of ICMR Task Force study, he studied on thalassemia in

India. The burden of haemoglobinopathies in India studied by Balgir (2000). Krishnamurti

(2000) in his report he observed the hemoglobin E-beta-thalassemia in Northeast India. Vaz

et al. (2000) finds the distribution of BT mutations in the Indian population. Piplani (2000)

observed the hemoglobin E disorders in North East India. Genetic epidemiology of the sickle

cell anaemia in India observed by Balgir (2001). Raj et al. (2001) studied an indigenously

manufactured rapid immunochromatographic test for detection of HBsAg. A screening test

for beta thalassemia trait pointed out by Mehta (2002). Agarwal et al. (2003) observed the

prenatal diagnosis in beta-thalassemia in India. Clinico-hematological profile of HbE

syndrome in adults and children studied by Tyagi et al. (2004). Safety of oral iron chelator

desferiprone in young thalassemics observed by Naithani et al. (2005).

Balgir (2005) in his ten years cohort study he studied the Spectrum of haemoglobinopathies

in the state of Orissa. Clinical and radiological study of oro-facial manifestations in

Thalassaemia studied by Patil (2006). Aberrant heterosis in haemoglobinopathies with special

reference to β -thalassemia and structurally abnormal haemoglobins E and S in Orissa, India.


48

find out by Balgir (2007). Bangal et al. (2009) observed the recurrent pregnancy loss due to

Haemoglobinopathy. Quais Mujawar et al. (2009) in his case study he reported haemoglobin

E-beta thalassemia at Bijapur, South India. Recently, Sharma and Pancholi (2010) studied the

management of TM by oral Iron Chelators.

Newborn Screening in India is studied by Seema Kapoor and Madhulika Kabra (2010). In

2010, Balgir found the phenotypic diversity of sickle cell disorders with special emphasis on

public health genetics in India. Singh et al., (2010) studied the effect of wheat grass tablets on

the frequency of blood transfusions in Thalassemia Major.

2.4 Transfusion transmitted Diseases

Orofacial complications in thalassemia

Pusaksrikit et al. (1987) finds the occlusion of the teeth in thalassemic patients. Drew and

Sach (1997) studied the management of thalassemia induced skeletal facial deformity. Effects

of thalassemia major on components of the craniofacial complex studied by Bassimitci

(1996). Agha (2000) evaluates the maxillofacial anomalies in Beta thalassemia major.

Hypoparathyroidism and intracranial calcification in b-thalassemia major observed by

Zafeiriou et al. (2001). Abu Alhaija et al. (2002) observed the cephalometric measurements

and facial deformities in subjects with beta-thalassaemia major. Singh and

Venketasubramanian (2004) studied the recurrent cerebral infarction in beta thalassaemia

major. Amini et al. (2007) studied the craniofacial morphology of Iranian children with TM.

Salehi et al. (2007) studied the prevalence of Orofacial complications in Iranian Patients with

β -Thalassemia Major. In 2008, Ashraf evaluates the oro-maxillofacial changes in major

thalassemia. Verma et al. (2007) studied the intracranial calcification in beta thalassemia

Major. Srdjan et al. (2008) observed the consanguineous marriages and endemic malaria, can

inbreeding increase population fitness.

Malaria in thalassemic patients

Nittis and Spiliopulos (1937) Studied that Mediterranean anemia may be a peculiar form of

malaria. A selective advantage for survival in individuals with the thalassemia trait in regions

where malaria is endemic. The RBCs of patients with Hb H disease have also shown a

suppressive effect on the growth of the parasites. This effect is not observed in α thalassemia

trait. Fawdry (1944) his studies focused on conducted Greeks and on Cyprus concluded that

malaria played no part in the causation of disease.


49

Dover and Schultz (1971) studied the transfusion-induced malaria. Transfusion-induced

malaria from an asymptomatic carrier observed by Najem and Sulzer (1976). In 1980, Joishy

and Lopez observed the transfusion-induced malaria in a splenectomized β-thalassemia major

patient and blood donor screening methods. Willcox et al. (1983) in his case study he

observed the Plasmodium falciparum malaria in haemoglobin S and beta-thalassemia traits.

Luzzi et al. (1991) finds the surface antigen expression on Plasmodium falciparum-infected

erythrocytes is modified in alpha- and beta-thalassemia. In 1993, Snounou et al. finds the

high sensitivity of detection of malaria parasites by the use of nested polymerase chain

reaction. Black et al. (1994) pointed out the mixed infections with Plasmodium falciparum

and P. malariae and fever in malaria. Williams et al. (1996) observed the high incidence of

malaria in alpha-thalassemic children.

Allen et al. (1997) studied the alpha -thalassemia protects children against disease caused by

other infections as well as malaria. Weatherall (1997) observed the correlation of the

thalassemia and malaria. Aluoch (1997) observed the higher resistance to Plasmodium

falciparum infection in patients with homozygous sickle cell disease in western Kenya. The

α+-thalassaemias are some of the best recognized malaria-protective polymorphisms. Flint et

al. (1998) studied the population genetics of the haemoglobinopathies. Williams (1999)

observes the mechanisms of malaria protection in the thalassemia syndromes.

Research involving both population and case-control studies has provided strong evidence

that the high frequency of the milder varieties of alpha-thalassemia is related to protection

against P. falciparum malaria (Weatherall and Clegg, 2002). Seed et al. (2005) reviewed the

status and potential role of laboratory testing to prevent transfusion-transmitted malaria.

Williams et al. (2005) observed the negative epistasis between the malaria-protective effects

of alpha+-thalassemia and the sickle cell trait. Kitchen and Chiodini (2006) observed the

blood transfusion transmitted malaria. Wambua et al. (2006) find out the effect of α +-

thalassemia on the Incidence of Malaria and other diseases in children living on the coast of

Kenya. May et al. (2007) studied the hemoglobin variants and disease manifestations in

severe P. falciparum. Transfusion-transmitted infections like hepatitis B, C, HIV, malaria and

syphilis studied by Hira et al, (2011).


50

Hepatitis/ Viral infections

Sumathy et. al, (1992) studied dipstick immunobinding enzyme-linked immunosorbent assay

for serodiagnosis of hepatitis B and delta virus infections. Ali et al. (2003) studied the

frequency of hepatitis C virus antibodies in blood donors in combined military hospital,

Quetta, at Pakisthan. Hepatitis C virus seropositivity in repeatedly transfused TM patients

studied by Muhammad et al. (2004). Zandieh et al. (2005) observed the Transfusion

Transmitted Virus (TTV) infection in thalassemic patients.

Spleenectomy

Cohen et. al (1980a) studied the transfusion requirements and splenectomy in thalassemia

major. Serum Levels of cytokines in poly-transfused patients with Beta-Thalassemia major:

Relationship to Splenectomy studied by Mohga et al. (2011). Al-Salem et al. (1989) studied

the splenectomy in children with sickle cell disease and thalassemia. Management of

thalassemia major by partial splenectomy studied by Bonani and Bahador (1994). Post-

splenectomy infection in Cooley’s anemia observed by Smith et al. (1964). Kheradpir MH,

Albouyeh M. Partial splenectomy in the treatment of thalassemia major studied by Kheradpir

et al. (1985). Moyamoya syndrome in a splenectomized patient with beta-thalassemia

intermedia observed by Sanefuji et al. (2006). Phrommintikul et al. (2006) observed the

Splenectomy and the risk factor for pulmonary hypertension in patients with thalassemia.

Other diseases and complications

Study of hepatitis B and C., prevalence and liver function in multiply transfused thalassemics

and their parents by author William et al. in 1992. Prati (2000) studied the benefits and

complications of regular blood transfusion in patients with beta-thalassemia major. In India

the study of fractures in transfusion dependent beta thalassemia by the author Basanagoudar

et al. (2001). Bruria et al. (2003) finds out the sleep disruption and objective sleepiness in

children with Thalassemia and congenital dyserythropoietic anemia. Lo et al. (2005)

observed the platelet alloimmunization after long-term red cell transfusion in transfusion-

dependent thalassemia patients. Masaya et. al (2010) studied the aortic valve replacement in a

patient with Alpha-Thalassemia. Kattamis et al. (1979) observed the chelation therapy and

ferritin levels in patients with homozygous β-thalassemia. Ghosh et al. (2000) he observed

the pseudomonas meningitis in adult, multitransfused thalassemia major patient.

Improvement in liver pathology of patients with β-thalassemia treated with Deferasirox for at

least 3 Years observed by Deugnier et al., (2011).


51

3. PURPOSE OF THE STUDY

Due to lack of education, awareness and information, thousands of children with thalassemia

are born each year and the number is growing day by day. No one can feel the pain and

sufferings except thalassemic children themselves and their parents. Thalassemic and their

parents are waiting for proper and uninfected blood transfusion, medication, laboratory tests

and clinical management needed to erase the shadow of miseries and hanging sword of death

on their heads.

The purpose of this study is to determine the extent of transfusion-induced complications like

transmition of malaria and other diseases like HCV, HBsAg, VDRL, HIV and

microorganisms among blood transfused thalassemic children from Solapur District,

Maharashtra State, India during August-2008 to July 2010.

A secondary purpose was to examine risk factors associated with blood transfusion in

thalassemiac patients. Possible risk factors are: gender, age, family history, genetic

classification, pathophysiology, symptoms, incidence of Alloimmunization in frequently

transfused thalassemic and to evaluate factors which may influence the development of

antibodies, transfusion transmitted diseases. In addition, other complications, medications,

and number of blood transfusions will be determined. For the purpose of this analysis,

patients were considered not at risk of malaria, other disease and complications after

receiving proper nutrition, time to time blood transfusion and medication.

The entire survey study is to was carried out under the observations of medical officer from

thalassemia transfusion centre, Indian Red Cross Society, Gopabai Damani Blood Bank,

Solapur Maharashtra, India. The present research work focused on awareness about

thalassemia, its prevention and its medical care. This study will helps to establish and develop

a collaborative network of national and international thalassemia affected individuals,

medical and scientific communities involved in the field; health-related organizations and

pharmaceutical industries.


52

4. OBJECTIVES

• To survey the thalassemic patients in different parts of Solapur district, Maharashtra

State, India.

• The aim of this thesis is to approach some of the existing problems related to the

scientific implementation of diagnosis, treatment and prevention by survey method,

possibly contributing to their solution.

• The current study aims to survey the effect of blood transfusion-induced or transmitted

malaria, other protozoan diseases and complications in thalassemia patient from Solapur

District.

• To avoid adverse reactions prevalence of infectious agents should safeguard the quality of

blood transfusion services. Blood donation practices, donor selection, and product

screening constitute some of the most important strategies that contribute to the safety

and adequacy of blood.

• The long term objective of this research is to develop in Solapur District, a National

referral center for blood transfusion in patients with thalassemia for proper monitoring of

better health care, to insist neonatal screening, regular laboratory tests, medication, proper

guidance for nutrition, health, awareness and proper education about the disease and

medication to the thalassemic patients countrywide.


53

5. RESULTS AND DISCUSSION

This study was done on 125 clinically proved cases of thalassemia. They were of the age

group between 6 months to 18 years, 73 being male and 52 female. They were from different

parts of Solapur District, Maharashtra State (Table 4 and 4a).

The present study was carried out in the following blood banks and hospitals.

1) Indian Red Cross Society, Gopabai Damani Blood Bank, Solapur.

2) Hedgewar Blood Bank, Solapur

3) Sou Sarjubhai Bajaj Blood Bank, Sub Branch Indian Red Cross Society, Pandharpur,

District-Solapur.

4) Shriman Rambhai Shah Blood Bank, Sub Branch, Indian Red Cross Society, Barshi,

District- Solapur.

5) Chatrapati Shivaji Rugnalaya, Government Hospital, Solapur.

Geographical distribution of thalassemia in Solapur District

The thalassemia patient’s code, patient’s initials, sex, diagnosis, Taluka wise distribution at

Solapur District, height, weight, growth and mortality shown in Table-4. The Taluka wise

geographical distribution of thalassemia patients and their prevalence percentage was shown

in Table-4a and Figure-3. The Talukawise distribution of thalassemia patients as follows:

Akkalkot: thalassemia major patients were five; total (4.00%)

Barshi: thalassemia major patients were five; total (6.40%)

Karmala: one BTI and TM three, total four patients (3.20%)

Madha: BTI three and TM two, total five patients (4.00%)

Malshirus: BTI one and TM three, total four (3.20%)

Mangalvedha: BTI two and TM four, Total six ((7.20%)

Mohol: BTMi two and TM four, total six (6.40%)

North Solapur: BTI one and TM two, total three (2.40%)

Pandharpur: BTI one, BTMi one and TM fifteen (12.00%)

South Solapur: BTI one, BTMi two and TM eleven, total fourteen (11.20%)

Sangola: BTMi one and TM four, Total five (4.80%)

Solapur city: SCT one, BTI one, TM 19, total forty two (35.20%).


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Table 4. Showes total number of thalassemia patient, with their code, initials, sex, diagnosis,

locality, height (Inch), weight (Kg), growth and mortality.

Patient

Code

Patient

Initials Sex Taluka Diagnosis

Height

(Inch)

Weight

(Kg)

Growth

Mortality

1 BMH F Akkalkot TM 24 7 Yes No

2 MAD M Mohol TM 24 8 Yes No

3 MPA F Solapur TM 24 8 Yes No

4 KPR F Solapur TM 26 9 Yes No

5 JSS F Solapur TM 25 9 Yes No

6 JP F S. Solapur TM 24 8 Yes No

7 GDD F S. Solapur TM 30 10 Yes No

8 DSS M Pandharpur BTI 34 8 Yes No

9 KLB F Solapur TM 32 10 Yes No

10 SGS M Malshirus TM 32 10 Yes No

11 MTM F Solapur TM 30 8 Yes No

12 PST M Solapur TM 36 12 No No

13 SSS M Mohol TM 32 10 Yes No

14 KSS M Solapur TM 34 12.5 Yes No

15 NA F Solapur TM 24 8.5 Yes No

16 MAR F Solapur TM 38 13.5 Yes No

17 STS F Sangola TM 38 13 Yes No

18 SSS M Solapur TM 34 15 Yes No

19 CRR M Akkalkot TM 32 10 Yes No

20 KSS M Solapur TM 40 12 Yes No

21 GSA M Solapur TM 41 20 No No

22 BSR F Pandharpur TM 37 12 Yes No

23 CNT M S. Solapur BTMi 34 12 Yes No

24 BKG F S. Solapur TM 36 10.5 Yes No

25 SOS1 M S. Solapur TM 37 13 Yes No

26 SOS2 M S. Solapur TM 36 12.5 Yes No

27 SSS M Malshirus TM 43 17 No No

28 BLM M N. Solapur BTI 42 15 No No

29 JPA F Solapur TM 37 14 Yes No

30 TRM M Mangalvedha TM 40 13 Yes No

31 PYR M Mangalvedha BTI 43 15 No No

32 RGS M Malshirus BTI 43 18.5 No No


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Table 4. Continued…

Code

Patient


Height

(Inch)

Weight

(Kg) Growth Mortality

33 MPP F Pandharpur TM 46 20 No No

34 GPO M Solapur TM 40 15 Yes No

35 TRI M Barshi TM 38 14 Yes No

36 KNS M N. Solapur TM 43 15 Yes No

37 VDS M Solapur TM 42 17 Yes No

38 PSB F Solapur BTMi 44 17.5 Yes No

39 RSB M Mangalvedha TM 42 16 Yes No

40 IVY F Pandharpur TM 41 14 Yes No

41 ARM M S. Solapur BTI 37 14 Yes No

42 GAR M Pandharpur TM 43 20 Yes No

43 KSM M S. Solapur TM 41 23 Yes No

44 KAV F Barshi TM 48 23 No No

45 PUA F Solapur TM 44 21 Yes No

46 DRS M Pandharpur TM 41 17 Yes No

47 BMN M Solapur TM 42 16 Yes No

48 LAB M Solapur TM 44 16 Yes No

49 VDS M Solapur TM 44 20 Yes No

50 SVP M Pandharpur BTMi 45 17.5 Yes No

51 KAN F Solapur TM 36 12 No No

52 SSS F Mohol TM 41 16 Yes No

53 BUT M Karmala TM 45 18 Yes No

54 MA M Sangola BTMi 36 20 Yes Yes

55 RSS M Mohol BTMi 44 20 Yes No

56 BNB M Solapur TM 43 15 Yes No

57 IAS M Solapur TM 45 15 Yes No

58 ISY M Pandharpur TM 44 13 Yes No

59 TAS M Madha BTI 48 15 No No

60 PAM F Solapur TM 45 20 Yes No

61 QSA M Karmala BTI 48 20 No No

62 BSR M Akkalkot TM 36 12 Yes Yes

63 RST F Solapur TM 45 16 Yes No

64 QAS F Karmala TM 47 22 Yes No


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Code

Patient


Height

(Inch)

Weight


65 GAM M Solapur TM 42 25 Yes Yes

66 PHM M Solapur TM 49 24 Yes No

67 GPS F Barshi TM 49 23 Yes No

68 SV M Solapur TM 36 15 Yes No

69 NDN F Pandharpur TM 47 25 Yes No

70 MLD M Solapur TM 49 23 Yes No

71 PPV F S. Solapur TM 53 24 No No

72 MVM M Madha TM 50 28 Yes No

73 KAS M Barshi TM 44 20 Yes No

74 CYS M Solapur SCT 50 19 Yes No

75 LS M Solapur TM 44 13 Yes Yes

76 GSS F Solapur TM 48 21 Yes No

77 CGR M S. Solapur TM 42 22 Yes No

78 RS M madha TM 44 22 Yes Yes

79 JSS F Akkalkot TM 46 20 Yes No

80 TNJ F S. Solapur TM 46 22 Yes No

81 MPT F Solapur TM 47 21 Yes No

82 BHH M Malshirus TM 48 25 Yes No

83 BSM M Solapur TM 54 30 No No

84 NRB F Pandharpur TM 53 28 Yes No

85 HNK M Akkalkot TM 52 28 Yes No

86 KVM M Mangalvedha BTI 45 33 Yes No

87 NSA M Sangola TM 48 28 Yes No

88 WAS M Solapur BTI 51 24 Yes No

89 NAP M Mangalvedha TM 52 27 Yes No

90 MSS M Sangola TM 50 25 No No

91 LSD M S. Solapur BTMi 53 27 Yes No

92 ISS F Solapur BTMi 51 22 Yes No

93 SAN F Mohol TM 45 20 Yes Yes

94 GSM F Solapur TM 48 20 Yes No

95 RKT F Solapur TM 49 21 Yes No


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Code

Patient


Height

(Inch)

Weight


96 RP M Pandharpur TM 44 22 Yes Yes

97 KSM M Pandharpur TM 58 28 Yes No

98 MRR F N. Solapur TM 52 20 Yes No

99 KPS F Solapur TM 59 30 No No

100 MMM F Barshi TM 54 26 Yes No

101 KBM F Mangalvedha BTI 48 23 Yes Yes

102 KJ F Solapur TM 48 20 Yes Yes

103 NPN F Pandharpur BTI 48 22 Yes Yes

104 KAA F Solapur TM 52 28.5 Yes No

105 PAD M Mohol TM 65 26.5 Yes Yes

106 HSD F Barshi TM 53 26 Yes Yes

107 GAS M Madha BTI 58 40 Yes No

108 KSS M Pandharpur BTI 58 35 Yes No

109 RYS M Sangola TM 51 26 Yes No

110 LSD F S. Solapur BTMi 63 45 No No

111 DML M Mohol BTMi 58 30 Yes No

112 SS M Pandharpur TM 58 32 Yes Yes

113 PKM M Barshi TM 52 28 Yes No

114 MGM M Madha BTI 54 31 Yes Yes

115 OBC F Karmala TM 55 29 Yes Yes

116 PVD M Mohol TM 65 36 Yes No

117 KKM F Mangalvedha BTI 62 35.5 No No

118 KR F Mangalvedha TM 48 30 Yes Yes

119 JVS M Sangola TM 60 30 Yes Yes

120 TAN F Solapur TM 60 38 No No

121 CLP F Solapur BTI 61 35 No No

122 DAM M Mangalvedha TM 48 39 Yes No

123 RVS M Solapur TM 56 30 Yes No

124 DRD F Barshi BTMi 56 30 Yes No

125 JSS F S. Solapur TM 60 33 No Yes


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Table 4a. Taluka wise geographical distribution and prevalence percentage of thalassemia

patients in Solapur District.

Parameter Sex SCT BTI BTMi TM Total Patients %

Akkalkot M 00 00 00 03 03

F 00 00 00 02 02

T 00 00 00 05 05(4.00)

Barshi M 00 00 00 03 03

F 00 00 00 05 05

T 00 00 00 08 08(6.40)

Karmala M 00 01 00 01 02

F 00 00 00 02 02

T 00 01 00 03 04(3.20)

Madha M 00 03 00 02 05

F 00 00 00 00 00

T 00 03 00 02 05(4.00)

Malshirus M 00 01 00 03 04

F 00 00 00 00 00

T 00 01 00 03 04(3.20)

Mangalvedha M 00 02 00 04 06

F 00 00 00 03 03

T 00 02 00 07 09(7.20)

Mohol M 00 00 2 04 06

F 00 00 00 02 02

T 00 00 02 06 08(6.40)

North Solapur M 00 01 00 01 02

F 00 00 00 01 01

T 00 01 00 02 03(2.40)

Pandharpur M 00 02 01 05 08

F 00 01 00 06 07

T 00 03 01 11 15(12.00)

South Solapur M 00 01 02 04 07

F 00 00 00 07 07

T 00 01 02 11 14(11.20)

Sangola M 00 00 01 04 05

F 00 00 00 01 01

T 00 00 01 05 06(4.80)

Solapur City M 01 01 00 19 21(28.76)

F 00 00 00 23 23(44.23)

T 01 01 00 42 44(35.20)


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Type of thalassemic patients in Solapur District

In our study, Sickle cell thalassemia was: male 1(1.36%), female 0(00%), Total 1(0.8%); BTI

was: male 12(16.43%), female 4(7.69%), Total 16(12.8%); BTMi was: male 06(8.21%),

female 4(7.69%), Total 10(8.0%); TM was: male 54(73.97%), female 44(84.61%), Total

98(78.4%); (Table- 4b and Figure- 4). The results compared the trequency of β-thalassemia

trait and other hemoglobinopathies in northern and western India with the reports of Nishi et

al., (2010). There was maximum number of 98(98.4%) cases of TM as compared to BTMi

10(8.0%), BTI 16(12.8%) cases and SCT 1(0.8%) was observed. No cases of α thalassemia

were observed in our research work.

Table- 4b. Showed type of thalassemic patients entering the study (n=125)

Type of thalassemic

patient

Sex

Number of

patients (%)

Prevalence %

SCT

M 1 0.80

F 0 0.00

Total 1 0.8

BTI

M 12 9.6

F 4 3.2

Total 16 12.8

BTMi

M 6 4.8

F 4 3.2

Total 10 8.0

TM

M 54 43.2

F 44 35.2

Total 98 78.4

Total patients

M 73 58.4

F 52 41.6

Total M+F 125 100


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Transfusion History:

All the 125 patients considered in our study were under regular blood transfusion therapy.

Age:

The age range of thalassemia patients (Table-4a) in our study was from 6 months to 18 years.

The mean age was around 8.75 years. Under age group 6 months-3 years, there were 19

male, 15 female patients and total male and female patients were 34(27.2%) observed. The

patients between 4-6 years age group was male 12, female 6 and total male and female

patients are 18(14.4%). In the age group of 7-10 years, the male patients are 22, female 11

and the total male and female patients were 33(26.4%) observed. The age group of 11-13

years, 9 male and 11 female patients observed, the total male and female patients were

20(16%). In the age group between 14-16 total 7 male and 3 female patients were observed,

the total male and female patients are 10(8%). In the age group between 17-18 years Male 4,

female 6 and total 10(8%) thalassemic patients were observed. Maximum patients [

34(27.2%)] were in the age group of 6 months to 3 years of age and minimum patients

[10(8%)] in both age group of 14-16 and 17-18 years age.

Sex:

Our study comprised of 73 (58.4%) males and 52(41.6%) females (Table- 4b) Thus, a higher

incidence of thalassemia in males was observed.

Height

The mean of Height of thalassemia patients showed in Table- 5 and Figure- 5. The mean

height of male patients were (45.43%) and female (44.67%). The total mean heigh of male

and female patients were (45.12%). The Height to Weight Ratio Chart is compared the data

from Disabled World. (2007).

Weight

The mean weight (Kg) of thalassemia patients showed in Table-5 and Figure-5. The average

mean weight of the male thalassemic patients were (20.45%) and female (20.38%) observed.

The total mean weitht of male and female patients were (20.42%). The observations of mean

height were showed the stunting of growth in thalassemic patients.


61

Table -5. Showed the mean height and weight in thalassemia patients.

Sex Male (%) Female (%) Total Male +

Female (%)

Mean height (Inch) 45.43 44.67 45.12

Mean weight (Kg) 20.45 20.38 20.42

Growth

The growth of thalassemic patients (Table-6 and Figure-6) was observed and compared with

Indian Standard Height Chart. Normal growth was: male (13.61%), female (19.23%) avarage

(16.00%); growth retardation was: male (86.30%), female (80%) average (84.00%) were

observed. TM is a serious medical problem. Growth retardation is commonly seen in poly-

transfused beta thalassemia patients. The exact mechanism of short stature in children with

thalassemia major is not well understood, however, it is believed to be multi-factorial. Thus,

it was observed that most of the thalassemic patients had poor build and demonstrated pallor.

In all patients, It was observed that the growth retardation was early in life, in association

with hypersplenism, poor musculature, and reduction of body fat, poor appetite and lethargy

observed. Neeraj et. al., (2010) their findings the present data comprises that the high proportion

of the patients show growth retardation.

Table -6. Showing prevalence percentage of growth in thalassemic patients.

Types of

Thalassemia

Sex

(Total patients)

Growth

Normal Growth failure

SCT

M (1) 0 1

F (0) 0 0

Total (1) 0 1

BTI

M (12) 5 7

F (4) 2 2

Total (16) 07 09

BTMi

M (6) 0 6

F (4) 1 3

Total (10) 01 09

TM

M (54) 5 49

F (44) 7 37

Total (98) 12 86

Total patients (%)

M (73) 10 (13.69) 63 (86.30)

F (52) 10 (19.23) 42 (80.76)

Total M+F (125) 20 (16.00) 105 (84.00%)


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Pedigree history of thalassemia patients

The pedigree of the 125 thalassemia patient and their families was represented in Figure-7.

Families are indicated with the name of the proband. Only initials of examined subjects were

reported. The observations in this pedigree support the hypothesis that the genes associated

with hemoglobin S, hemoglobin C and "classical thalassemia" are either very closely linked

or true alleles. The pedigree can identify family members who may be at risk of having

thalassemia disease or trait and should therefore be tested. A genetic counselor may also

identify a history of other conditions that may have a genetic component.


63

Figure-7. Pedigree of thalassemia patient’s family.

Note: Number indicates patient’s case number and initials indicate patient’s names.

1 BMH

7 GDD

2 MAD

8 DSS

3 MPA

9 KLB

4 KPR

10 SGS

5 JSS

11 MTM

6 JP

12 PST


64

Figure-7. Continued..

13 SSS

19 CRR

14 KSS

20 KSS

15 NA

21 GSA

16 MAR

22 BSR

17 STS

23 CNT

18 SSS

24 BKG


65


25 SOS1

31 PRY

26 SOS2

32 RGS

27 SSS

33 MPP

28 BLM

34 GPO

29 JPA

35 TRI

30 TRM

36 KNS


66


37 VDS

43 KSM

38 PSB

44 KAV

39 RSB

45 PUA

40 IVY

46 DRS

41 ARM

47 BMN

42 GAR

48 LAB


67


49 VDS

55 RSS

50 SVP

56 BNB

51 KAN

57 IAS

52 SSS

58

53 BVT

59 TAS

54 GHS

60 PAM


68


61 QSA

67 GPS

62 BSR

68 SV

63 RST

69 NDN

64 QAS

70 MLD

65 GAM

71 PPV

66 PHM

72 MVM


69


73 KAS

79 JSS

74 CYS

80 TNJ

75 LS

81 MPT

76 GSS

82 BHH

77 CGR

83 BSM

78 RS

84 NRB


70


85 HNK

91 LSD

86 KVM

92 ISS

63 SAN

87 NSA

93 SAN

88 WAS

94 GSM

89 NAP

95 RKT

90 MSS

96 RP


71


97 KSM

103 NPN

98 MRR

104 KAA

99 KSP

105 PAD

100 MMM

106 HSD

101 KBM

107 GAS

102 KJ

108 KSS


72


109 RYS

115 OBC

110 LSD

116 PVD

111 DML

117 KKM

112 SS

118 KP

113 PKM

119 JVS

114 MGM

120 TAN


73


121 CLP

124 DRD

122 DAM

125 JSS

123 RVS


74

Education

The literacy percentage (Table-7 and Figure-8) in thalassemia patients was: educated male

(65.75%), female (63.46%) avarage (64.8%); below four years of age- male (8.21%), female

(19.23%) avarage (12.8%); uneducated male (26.02%), female (17.30%) avarage (22.4%).

The majority (>22.4%) of thalassemia patients were illiterate with only 1.6% with a higher

education. General health education for those, suffering from thalassaemia, which will help

prevention and spread of thalassemia (Qamruz and Salahuddin, 2006). The present data

analyzed with the reports of Pakbaz et al., (2010), he observed the education and employment

status of children and adults with thalassemia in North America. Due to lack of education,

awareness and information, thousands of children with thalassemia are born each year and the

number is growing day by day. No one can feel the pains of sufferings except thalassemic

children themselves and their parents. Thalassemic and their parents are waiting for proper

and uninfected blood transfusion, medication, laboratory tests and clinical management

needed to erase the shadow of miseries and hanging sword of death on their heads.

Table-7. Showing the education in thalassemic patient

Sex Class Male Female Total Male + Female (%)

Education

LKG 2 2 4

UKG 1 1 2

1st 10 1 11

2nd 3 5 8 3rd 7 2 9

4th 4 1 5 5th 3 7 10

6th 4 7 11

7th 6 1 7

8th 0 3 3

9th 3 1 4 10th 5 2 7

11th 0 2 2 12th 0 0 0

B.A -I 0 1 1

*Below 4 Yr Total %

6 10 16

Uneducated Total %+ Below 4 Yr

19 + *6 = 25

9 +*10 =19 28 + *16 =44

Educated Total % 48 (65.75) 33 (63.46) 81 (64.8)

*Below 4 years patients considered as uneducated


75

Skin coloration

The skin color change (Table- 8 and Figure- 9) in thalassemia patients was: fair male

(23.28%), female (26.92%) avarage (24.8%); wheatish male (53.42%), female (59.61%)

avarage (56.0%); brownish male (4.10%), female (0.00%) total (2.4%); blackish male

(19.1%), female (13.46%) avarage (16.8%) were observed.

Table- 8. Showing the skin color of thalassemic patient.

Sex Male (%) Female (%) Total Male +

Female (%)

Skin colour

Fair 17 (23.28) 14 (26.92) 31(24.8)

Wheatish 39 (53.42) 31 (59.61) 70 (56.0)

Brownish 3 (4.10) 0 (0.00) 3 (2.4)

Blackish 14 (19.1) 7 (13.46) 21 (16.8)

Hearing and visual impairment

There was no hearing and visual impairement (Table-9 and Figure-10). Due to proper diet,

medication and regular blood transfusion, these complications are not seen in all thalassemia

patients.

Psychological impaired

The psychological study of all thalassemia patients showed in (Table-9 and Figure-10). In

the present observations all male patients were normal (100%), in female only one was

psychological impaired. In India Atika et al. (2006) observed the psychosocial problems in

thalassemic childrens and their families. It is evident from the findings of our study that

besides the clinical burden, thalassemic have to shoulder very low psychosocial burden

associated with the disease. There was no any correlation between the psychological burden

and the sex.

Table-9. Showing hearing, visual and psychological complications in thalassemia patients.

Sex Parameter Male (%) Female (%) Total Male +

Female (%)

Hearing Normal 73 (100.0) 52 (100.0) 125 (100.0)

Ophthalmological Normal 73 (100.0) 52 (100.0) 125 (100.0)

Psychology

Normal 73 (100.0) 51 (98.7) 124 (99.2)

Abnormal 00 (0) 01(1.92) 1 (0.8)


76

Consanguineous marriage:

Consanguineous means having similar blood. A marriage between close relatives is referred

to as a consanguineous marriage. The cases of consanguineous marriage (Table-11 and

Figure-11) in thalassemia patients were: male parents 33(45.20%), female parents

30(57.69%) total 63(50.4%); Non consanguineous marriage were observed in male parents

40(54.79%), female parents 22(42.30%) total 62(49.6%). The observations showed the

consanguinity rate was very high (>49%) in both male and female. The results indicate the

need for implementing a comprehensive genetic preventive program for the eradication of

thalassemia in Solapur District. Neeraj et. al. (2010), he observed the consanguinity

marriages of thalassemic patients in Muslim and Sindhi religion. The observations showed the

consanguinity rate was very high (>49%) in male and female. The results indicate the need for

implementing a comprehensive genetic preventive program for the eradication of thalassemia in

Solapur District.

Table 11. Showing prevalence percentage of the consangunous marriages in the parents of

the thalassemic patient.

Sex Male (%) Female (%) Total Male + Female (%)

Consanguinity

Yes 33 (45.20) 30 (57.69) 63 (50.4)

No 40 (54.79) 22 (42.30) 62 (49.6)

Ethnicity

Religion wise percentage of thalassemia (Table-12 and Figure-12). It was very high in

Hindu and muslin as compared to Jain and Sindhi community.

Table-12. Showing the percentage of ethnicity in thalassemic patients.

Ethnicity Male (%) Female (%) Total Male + Female (%)

Hindu 59 (80.82) 42 (80.76) 101 (80.8)

Jain 1 (1.36) 00 (0.00) 1 (0.8)

Muslim 12 (16.43) 10 (19.23) 22 (17.6)

Sindhi 1 (1.36) 00 (0) 1 (0.8)

Diet

There was no any correlation between the diet and sex (Table-13 and Figure-13).


77

Table-13.. Showing prevalence percentage of thalassemic patients’ diet.

Sex Diet Male (%) Female (%) Total Male + Female (%)

Diet

Vegetarian 23 (31.50) 20 (38.46) 43 (34.4)

Non vegetarian 50 (68.49) 32 (61.53) 82 (65.6)

Orofacial complications

The prevalence of orofacial complications (Table-14) in Thalassemia patients was: rodent

face (Figure-14) male (73.97%), female (75.00%) total (74.4%); saddle nose (Figure-14a)

male (79.45%), female (90.38%) total (84.00%); maxillary protrusion (Figure-14b) male

(79.45%), female (82.69%) total (80.8%); Upper anterior teeth spacing (Figure-14c) male

(52.05%), female (53.84%) total (52.8%); Anterior open bite (Figure-14d) male (69.86%),

female (78.84%) total (73.6%); deep bite (Figure-14e) male (90.41%), female (1.92%) total

(53.6%); Mucosal discoloration (Figure-14f) male (0.00%), female (1.92%) total (0.8%);

respectively. In this study, Upper anterior teeth spacing and deep bite was observed, more

than the normal population especially in older patients, which is due to rotation of mandible

and protrusion of maxilla leading to over growth of upper anterior teeth of maxilla.

The anterior open bite was slightly more than normal population. Regular and repeated

blood-infusion keeping the hemoglobin in an appropriate level (at least 10g/dl), along with

iron removal can prevent face and skull deformities. There was not any relationship between

the complications and sex. Skull and face deformities can be closely related to the patient's

age. Most of the patients were in the first and second decade of life. Early diagnosis and

blood transfusion caused less prevalence of rodent face. The orofacial complications in

patients with thalassemia major were similar to findings of other studies (Drew and Sach,

1997; Agha and Shabandy, 2000). The results of orofacial complications in thalassemic

patients showed that the rate of rodent face, maxillary protrusion, Anterior open bite, and

saddle nose increased with age. This was quite predictable because the maxilla is one of the

most common bones affected by thalassemia (Kliegman and Behromon, 2002). The

evaluation of oro-maxillofacial changes in major thalassemia observations were similar to

results reported by Salehi (2007) and Ashraf et al., (2008). Overall, it indicated a reduction in

complications during last decades, which was due to early diagnosis, treatment, and regular

follows up. The orofacial complications, the data was corroborated with the reports of of

Kremer (1986).


78

Table-14. Showing the sex wise prevalence of orofacial complications in thalassemic

patients.

Parameter Sex Yes

SCT

No

SCT

Yes

BTI

No

BTI

Yes

BTMi

No

BTMi

Yes

TM

No

TM

Yes

(%)

No

(%)

Rodent

face

M 0 1 9 3 5 1 40 14 54

(73.97)

19

(26.02)

F 0 0 3 1 4 0 32 12 39 (75)

13 (25)

T 0 1 12 4 9 1 72 26 93 (74.4)

32 (25.6)

Saddle nose

M 0 1 11 1 6 0 41 13 58 (79.45)

15 (20.54)

F 0 0 3 1 3 1 41 3 47 (90.38)

5 (9.61)

T 0 1 14 2 9 1 82 16 105 (84)

20 (16)

Maxillary protrusion

M 0 1 11 1 6 0 41 13 58 (79.45)

15 (20.54)

F 0 0 4 0 3 1 36 8 43 (82.69)

9 (17.30)

T 0 1 15 1 9 1 77 21 101

(80.8)

24

(19.2)

Maxillary

anterior

teeth

spacing

M 0 1 5 7 3 3 30 24 38

(52.05)

35

(47.94)

F 0 0 4 0 2 2 22 22 28

(53.84)

24

(32.87)

T 0 1 9 7 5 5 52 46 66

(52.8)

59

(47.2)

Anterior

open bite

M 0 1 10 2 6 0 35 19 51

(69.86)

22

(30.13)

F 0 0 4 0 2 2 35 9 41

(78.84)

11

(21.15)

T 0 1 14 2 8 2 70 28 92

(73.6)

33

(26.4)

Deep bite

M 0 1 1 11 0 6 6 48 66 (90.41)

7 (9.58)

F 0 0 0 4 0 4 1 43 1 (1.92)

51 (98.07)

T 0 1 1 15 0 10 7 91 67 (53.6)

58 (46.6)

Mucosal

discolorati

-on

M 0 1 0 12 0 6 0 54 0

(0)

73

(100)

F 0 0 0 4 0 4 1 43 1

(1.92)

51

(98.07)

T 0 1 0 16 0 10 1 97 1

(0.8)

124

(99.2)


79

Hematological Parameters

Hematological parameters: WBC count, RBC count, Hb, HCT, MCV, MCH, HCHC and

platelets count were measured using Sysmex cell counter (Wild and Bain, 2001).

RBC morphology

In present survey it was observed that morphology of RBC were abnormal in all 125

thalassemic patients. The morphology of RBC was showed in Figure-15. The following

types of the RBC morphology were observed:

1) Hypochromasia: Red blood cell with a decreased concentration of hemoglobin

2) Macrocytosis: Larger than normal red blood cell

3) Microcytosis: Decrease in the red cell size. Red cells are smaller than ± 7µm in diameter.

4) Anisocytosis: RBC are of unequal size.

5) Poikilocytosis:

a) Target cells: Red cells have an area of increased staining which appears in the area of

central pallor.

b) Ovalocytes: oval shape red blood cell

c) Tear drop cells: Red cells are shaped like a tear drop or pear.

d) Acanthocytosis: RBCs with irregularly spaced projections, these projections very in

width but usually bear a rounded end. These types of cells were observed in splenectomized

thalassemic patients.

e) Sickle Cells: Sickle shaped red cells. Only one male patient was observed sickle cell beta

thalassemia (Hb-S disease).

6) Elliptocytosis: RBCs are oval or elliptical in shape. Long axis is twice the short axis.

7) Envelope form cell: Some of RBCs are overlapped.

8) Basophilic stippling: Large numbers of small basophilic inclusions in RBCs.

WBC, RBC, Hb, HCT, MCV, MCH, MCHC, platelets and Alloimmunization

The normal reference value was showed in Table-15. Hematological parameters were

observed by using cell counter (Sysmex KX21), the report showed in the Table-15.


80

Table-15. Showing the normal reference value of WBC, RBC, Hb, HCT, MCV, MCH,

HCHC and Platelets.

Parameters Age Normal range male Normal range female

WBC 1 Year

2-18 Years

6-18x103/µl

3.5-11x103/µl

6-18x103/µl

3.5-11x103/µl

RBC 6-11 months

1-11 Years

12-18 Years

3.9-5.5 x106/µl

3.8-4.8 x106/µl

4.1-5.2 x106/µl

3.9-5.5 x106/µl

3.8-4.8 x106/µl

4.1-5.0 x106/µl

Hb 6 months-8 Years

9-11 Years

12-18 Years

11.2-14.1g/dl

12-15g/dl

11.7g/dl

12.2-14.1g/dl

12-15g/dl

11.5-15.3g/dl

HCT 6 months to 18 Years 45-62% 37-48%

MCV 6 months-8 Years

9-11 Years

12-18 Years

68-85 fl

75-87 fl

77-95 fl

68-85 fl

75-87 fl

77-95 fl

MCH

6 months-8 Years

9-11 Years

12-18 Years

24-30 pg

26-32 pg

27-34 pg

24-30 pg

26-34 pg

27-34 pg

MCHC

6 months- 11Years

12-18 Years

32-37 g/dl

32-37 g/dl

32-37 g/dl

32-36 g/dl

Platelets 6 months to 18 Years 1.5-4.5x103/µl 1.5-4.5x10

3/µl


81

Table-15. Showing the hematological parameters in the thalassemic patients.

Patient’s

Code

WBC X

103 /

µl

RBC

X106 /

µl

HGB X

g/ dl

HCT

% MCV fl

MCH

pg

MCHC

g/dl

Platelet

X103 /µl

Alloimmu

-nization

1 15.76 3.88 10.06 29.2 75.63 26.16 34.56 551.66 Neg

2 6.76 3.07 7.7 22.83 74.5 25.2 33.83 416.33 Neg

3 6.76 2.61 6.16 18.8 72 23.63 32.86 95.33 Neg

4 7.5 3.36 8.26 24.4 73 24.73 33.83 322 Neg

5 14.36 3.97 10.83 31.23 78.63 27.26 34.66 580 Neg

6 6.93 3.62 6.16 26.56 73.43 24.96 34 263.33 Neg

7 6.26 2.89 7.46 21.2 77.53 26.1 33.63 278.33 Neg

8 9.1 3.91 10.06 29.86 76.16 25.66 33.63 294.33 Neg

9 5.93 2.89 7.06 21.26 75.13 25.16 33.4 343.33 Neg

10 15.9 3.03 7.53 22.8 74.86 24.7 32.9 652 Neg

11 5.2 2.42 5.9 18.1 74.7 24.6 32.9 147.66 Neg

12 4.56 2.59 6.4 19.56 75.9 25 32.86 284 Neg

13 9.63 3.11 7.66 22.56 72.43 24.6 33.96 390.66 Neg

14 17.2 2.5 6.46 19.6 78.53 25.96 33.1 478 Neg

15 12.1 2.69 7.46 21.43 79.46 27.7 34.9 485.66 Neg

16 6.2 2.58 6.66 20 77.56 25.83 33.3 262.66 Neg

17 10.7 3.19 8.63 25.76 80.63 26.93 33.43 385.66 Neg

18 17.8 3.65 10.1 29.4 79.96 27.43 34.36 477 Neg

19 5.8 2.92 6.7 22.96 73.13 23.16 31.7 185.33 Neg

20 9.9 3.73 8.73 26.96 72.3 23.36 32.4 240 Neg

21 11.1 2.79 8.83 22.96 81.9 31.1 37.9 472.66 Neg

22 6.66 3.15 7.3 22.9 72.4 23.03 31.8 386.66 Neg

23 14.7 2.7 7.1 20.46 75.76 26.36 35.13 377.33 Neg

24 7.96 2.96 7.66 22.73 76.33 18.43 33.73 309.66 Neg

25 13.1 3.3 8.56 25 75.66 25.93 34.26 259.66 Neg

26 10.2 2.77 7.6 21.86 78.73 27.36 34.73 164 Neg

27 5.63 2.87 7.26 21.7 77.36 25.86 33.46 293.66 Neg

28 10 3.83 9.93 29.26 76.53 25.96 33.93 341 Neg

29 7.76 3.51 8.3 25.7 72.56 23.36 29.53 286.66 Neg

30 6.93 3.65 9.5 27.5 75.2 25.96 34.56 345 Neg

31 8.76 3.2 8.43 25.46 79.63 26.36 33.1 371.66 Neg


82

Table-15. Continued..

Patient’s

Code

WBC X

103 /

µl

RBC

X106 /

µl

HGB X

g/ dl

HCT

% MCV fl

MCH

pg

MCHC

g/dl

Platelet

X103 /µl

Alloimmu

-nization

32 6.3 3.8 7.33 24.23 63.7 19.26 30.23 344.66 Neg

33 10.26 3.38 8.66 25.9 77.86 26.1 33.5 169.66 Neg

34 6.76 2.6 6.6 19.83 76.03 25.23 33.45 167.33 Neg

35 6.26 3.62 9.53 28.5 78.5 26.2 33.4 128.33 Positive

36 7.46 2.98 8.16 23.76 79.43 27.26 34.33 276 Neg

37 10.6 3.21 8.86 27.86 80.8 27.63 34.2 249 Neg

38 5.63 2.87 7.26 21.7 77.46 25.86 33.46 293.66 Neg

39 13.96 3.6 9.43 28.7 79.56 26.13 32.86 283.66 Neg

40 8.2 3.09 8.03 24.06 77.93 26.06 33.46 300 Neg

41 6.93 3.84 9.83 29.5 76.8 25.6 33.3 270 Neg

42 9.4 3.54 8.1 25.3 71.23 22.76 28.6 267.66 Neg

43 8.3 3.47 8.23 25.23 72.63 23.73 32.63 322.66 Neg

44 4.43 3.46 7.9 24.8 71.56 22.8 31.8 339.66 Neg

45 41.4 3.06 7.63 23.66 77.3 24.93 32.23 624 Neg

46 6.16 3.28 8.53 25.73 78.63 26.1 33.1 324.66 Neg

47 6.2 2.49 6.2 18.33 78.66 26.7 33.9 270.66 Neg

48 10.06 3.31 8.73 25.83 77.9 26.2 33.66 269 Neg

49 8.5 2.66 7.26 21.03 78.8 27.23 34.56 298.66 Neg

50 8.56 3.02 8.06 23.93 79.26 26.7 33.66 373.33 Neg

51 7.26 2.83 8.13 22.9 80.73 28.5 35.2 309.33 Neg

52 6.33 2.84 6.96 21.3 74.7 24.43 32.66 170 Neg

53 5.43 2.56 6.8 20.16 78.76 26.6 33.76 307.66 Neg

54 6.36 3.48 9.43 26.73 76.83 27.16 35.3 289 Neg

55 8.86 3.13 8.4 26.2 81.33 26.83 33 236 Neg

56 7.23 2.85 7.16 21.73 76.2 25.13 33.03 246.33 Neg

57 7.7 3.98 11.23 33.63 83.73 28.06 33.53 131.33 Neg

58 8 3.25 8.23 23.9 73.5 25.3 34.3 388.33 Neg

59 8.46 4.17 10.33 30.6 73.36 24.76 33.76 207.66 Neg

60 6.43 2.81 7.7 22.56 80.13 27.36 34.13 407.66 Neg

61 5.3 2.64 6.76 20.23 76.13 25.36 33.26 193.66 Neg

62 5.7 3.35 9.03 25.3 75.6 25.3 35.76 271 Neg

63 14.26 3.69 9.56 29 78.73 25.93 32.96 490 Positive

64 23.9 3.49 9.13 28.03 80.36 26.2 32.6 530.33 Neg

65 13.46 3.22 8.43 25.56 79.26 26.13 33.03 1014 Neg


83


Patient’s

Code

WBC X

103 /

µl

RBC

X106 /

µl

HGB X

g/ dl

HCT

% MCV fl

MCH

pg

MCHC

g/dl

Platelet

X103 /µl

Alloimmu

-nization

66 11.03 2.77 7.43 21.83 78.7 26.83 34.06 445.66 Neg

67 4.63 3.68 9.26 28.23 76.73 25.16 32.83 267.33 Neg

68 7.5 3.18 8.16 24.2 76.36 25.8 33.76 305.33 Neg

69 8.3 3.1 8.6 26.4 85 27.73 32.56 423.16 Neg

70 4.56 2.6 7.53 22.66 86.93 28.83 33.13 301.66 Neg

71 7.73 2.78 6.86 21.16 76.36 24.7 32.3 244 Neg

72 6.1 2.91 7.16 21.7 74.33 24.7 33.16 259.66 Neg

73 10.3 3.24 8.03 24.7 75.03 24.36 32.43 396.66 Neg

74 7.1 3.34 8.46 25.16 75.43 25.43 33.66 284 Neg

75 24.56 2.26 6.7 19.3 85.36 29.7 34.86 510.33 Neg

76 5.83 3.27 9.1 27.26 83.1 27.8 33.46 207.66 Neg

77 8.3 2.84 6.9 20.46 72.4 24.53 34.6 537.66 Neg

78 5.56 2.86 6.66 19.4 72.5 23.43 32.33 161 Neg

79 8.76 2.99 7.53 22.93 77.16 25.43 32.9 269.33 Neg

80 4.83 2.33 6.3 18.53 79.3 26.93 33.9 280 Neg

81 7.33 2.97 7.63 23.26 78.6 25.8 32.83 417.33 Neg

82 6.5 2.8 7.23 21.76 77.8 25.86 38.23 187.66 Neg

83 2.46 4.15 10.96 31.53 76.6 26.36 34.7 157.33 Neg

84 6.56 2.92 6.7 21.3 73.6 19.4 31.56 358 Neg

85 7.76 2.11 4.8 15.23 72 7.4 21.5 137.66 Neg

86 7.7 3.34 8.3 25.06 75.03 24.9 33.1 443.33 Neg

87 7.76 3.35 7.2 23.43 69.63 21.53 30.7 374.33 Neg

88 4.66 2.33 6.13 18.8 80.2 26.06 32.5 234.33 Neg

89 14.06 2.93 7.93 24 81.33 26.86 33.1 208.66 Neg

90 15.4 2.95 8.1 24.5 82.4 26.5 32.6 702 Neg

91 4.46 2.3 6.73 20.8 91 29.33 32.26 233.66 Neg

92 5.76 2.39 6 18.33 76.96 25.2 32.7 174.33 Neg

93 6.16 2.08 5.7 16.83 80.73 27.26 33.8 94 Neg

94 5.76 2.7 6.8 20.3 75.76 25.1 33.13 250.33 Neg

95 5.26 3.35 6.13 18.36 70.86 23.53 33.13 194 Neg

96 7.36 3.38 8.66 24.86 73.66 25.63 34.66 261 Neg

97 6.8 2.85 7.93 23.13 80.86 27.73 34.33 237 Neg

98 5.2 3.45 7.5 23.66 68.4 21.66 31.7 342.33 Neg


84


Patient’s

Code

WBC X

103 /

µl

RBC

X106 /

µl

HGB X

g/ dl

HCT

% MCV fl

MCH

pg

MCHC

g/dl

Platelet

X103 /µl

Alloimmu

-nization

99 18.43 3.2 8.13 25.46 79.63 25.43 30.8 1027 Neg

100 6.3 2.36 6.33 19.36 81.9 26.76 32.73 151.66 Neg

101 7.7 3.5 9.03 27.03 76.06 25.5 33.43 382 Neg

102 5.36 2.71 7.16 20.5 75.73 26.5 34.9 186 Neg

103 26.3 2.46 7.06 20.8 84.56 28.73 33.96 471.33 Neg

104 4.83 2.8 7.26 21.7 78.33 26.2 33.5 329.33 Neg

105 25.6 2.67 6.3 20.66 77.4 23.5 30.33 686.66 Neg

106 15.9 2.79 7.53 23.3 82.93 26.9 32.36 898 Neg

107 9.73 4.06 9.06 28.4 68.33 22.33 32.73 126.33 Neg

108 4.16 2.01 5.33 16.5 82.06 26.56 32.33 177.33 Neg

109 29.96 2.59 7.13 21.9 84.7 27.63 32.6 332 Neg

110 4.8 2.62 6.9 21.5 82.16 26.36 32.06 255.33 Neg

111 7.1 2.92 7.5 22.5 77.46 26.6 33.6 321.33 Neg

112 24.8 2.36 7 19.96 84.6 29.66 35.06 489.66 Neg

113 5.5 2.89 7.23 22.1 76.76 25.13 32.73 129 Neg

114 5.5 2.45 5.76 17.73 72.4 23.56 32.5 274 Neg

115 5.53 2.13 5.53 16.63 78.1 25.9 33.16 201.66 Neg

116 21.83 3.21 8.4 26.1 81.5 26.23 32.2 748 Neg

117 7.5 3.01 7.36 22.3 74.1 24.43 32.96 271.66 Neg

118 5.4 3.09 8.16 22.8 74.16 26.73 35.86 199.33 Neg

119 27.4 2.69 7.1 21.93 81.83 26.5 32.33 868 Neg

120 4.03 2.31 5.73 11.03 77.93 24.73 31.73 201.66 Neg

121 23.66 2.44 6.7 20.53 84.36 27.56 32.66 692.66 Neg

122 4.6 2.77 6.66 20.8 78.2 25.06 32.6 220 Neg

123 17.4 3.13 8 24.56 78.46 25.53 32.56 549.33 Neg

124 3.06 1.86 4.8 14.76 79.33 25.8 32.5 105.33 Neg

125 12.1 3.43 9.16 18.46 78.06 26.66 34.23 862.66 Neg


85

WBC Count

The WBC counts of thalassemic patients were showed in the Table-16 and Figure-16 . The

low WBC count was not found in any patients. The normal WBC was 80.80% in male and

female, high WBC count was 19.20% in male and female patients.

Table-16. Showing the prevalence percentage of WBC count in thalassemia patients.

Types of

Thalassemia

Sex

(Total patients)

WBC count (%)

Normal (%) High (%) Low (%)

SCT

M (1) 1(100.00) 00(00.00) 00(00.00)

F (0) 00(00.00) 00(00.00) 00(00.00)

Total(1) 1(100.00) 00(00.00) 00(00.00)

BTI

M (12) 12 (100.00) 00(00.00) 00(00.00)

F (4) 2(50.00) 2(50.00) 00(00.00)

Total (16) 14(87.50) 2 (12.50) 00(00.00)

BTMi

M (6) 5(83.30) 1(16.60) 00(00.00)

F (4) 4(100.00) 00(0.00) 00(00.00)

Total (10) 9(90.00) 1(10.00) 00(00.00)

TM

M (54) 39(72.22) 15(27.77) 00(00.00)

F (44) 38(86.36) 6(13.63) 00(00.00)

Total (98) 77(78.57) 21(21.42) 00(00.00)

Total patients %

M (73) 57(78.08) 16(21.91) 00(00.00)

F (52) 44(84.61) 08(15.38) 00(00.00)

Total M+F (125) 101(80.80) 24(19.20) 00(00.00)


86

RBC Count

The RBC count was showed in Table-17 and Figure-17. In all 125 thalassemia patients,

normal RBC count was: male (6.84%), female (1.92%) avarage (4.80%); high RBC count

was not fount in either sex; low RBC count was male (93.15%), female (98.06%) total

(95.2%). Red cells carry oxygen to the tissues, when RBCs are low, the body does not get

enough oxygen and a person may feel tired or short of breath, heart beat faster than normal.

In all majority thalassemia patient’s skin color may be pale due to very low range of red

blood cells. The results shows that the absent beta globin chain synthesis, resulting in reduced

Hb in red blood cells (RBC), decrease RBC production leading to anemia.

Table-17. Showing the RBC count in thalassemia patients.

Types of

Thalassemia

Sex

(Total patients)

RBC (%)

Normal High Low

SCT

M (1) 00(00.00) 00(00.00) 01 (100.00)

F (0) 00(00.00) 00(00.00) 00(00.00)

Total (1) 00(00.00) 00(00.00) 01(100.00)

BTI

M (12) 03(25.00) 00(00.00) 09 (75.00)

F (4) 00(00.00) 00(00.00) 04(100.00)

Total (16) 03(18.75) 00(00.00) 13(81.25)

BTMi

M (6) 00(00.00) 00(00.00) 06(100.00)

F (4) 00(00.00) 00(00.00) 04(100.00)

Total (10) 00(00.00) 00(00.00) 10(100.00)

TM

M (54) 02(3.84) 00(00.00) 52(96.29)

F (44) 01(2.27) 00(00.00) 43(97.72)

Total (98) 03(3.06) 00(00.00) 95(96.93)

Total patients %

M (73) 05 (6.84) 00(00.00) 68(93.15)

F (52) 01(1.92) 00(00.00) 51(98.06)

Total M+F (125) 06(4.80) 00(00.00) 119(95.2)


87

Hemoglobin level

The Hb level was shown in Table-18 and Figure-18. In all type of thalassemic patients Hb

level was very low. Not a single patient reported normal or high Hb.

Table-18. Showing the prevalence percentage of Hb in thalassemic patients.

Types of

Thalassemia

Sex

(Total patients)

Hb (%)

Normal High Low

SCT

M (1) 00(00.00) 00(00.00) 1(100.00)

F (0) 00(00.00) 00(00.00) 00(00.00)

Total 00(00.00) 00(00.00) 01(100.00)

BTI

M (12) 00(00.00) 00(00.00) 12(100.00)

F (4) 00(00.00) 00(00.00) 04(100.00)

Total (16) 00(00.00) 00(00.00) 16(100.00)

BTMi

M (6) 00(00.00) 00(00.00) 06(100.00)

F (4) 00(00.00) 00(00.00) 04(100.00)

Total (10) 00(00.00) 00(00.00) 10(100.00)

TM

M (54) 00(00.00) 00(00.00) 54(100.00)

F (44) 00(00.00) 00(00.00) 44(100.00)

Total (98) 00(00.00) 00(00.00) 98(100.00)

Total patients

M (73) 00(00.00) 00(00.00) 73(100.00)

F (52) 00(00.00) 00(00.00) 52(100.00)

Total M+F (125) 00(00.00) 00(00.00) 125(100.00)


88

HCT (Hematocrit or Pack cell volume)

The HCT level was shown in Table-19 and Figure-19. In all type of thalassemic patients

HCT count was very low. Not a single patient was with normal or with high HCT.

Table-19. Showing the prevalence percentage of HCT count in thalassemia patients.

Types of

Thalassemia

Sex

(Total patients)

HCT (%)

Normal High Low

SCT

M (1) 00(00.00) 00(00.00) 1(100.00)

F (0) 00(00.00) 00(00.00) 00(00.00)

Total 00(00.00) 00(00.00) 01(100.00)

BTI

M (12) 00(00.00) 00(00.00) 12(100.00)

F (4) 00(00.00) 00(00.00) 04(100.00)

Total (16) 00(00.00) 00(00.00) 16(100.00)

BTMi

M (6) 00(00.00) 00(00.00) 06(100.00)

F (4) 00(00.00) 00(00.00) 04(100.00)

Total (10) 00(00.00) 00(00.00) 10(100.00)

TM

M (54) 00(00.00) 00(00.00) 54(100.00)

F (44) 00(00.00) 00(00.00) 44(100.00)

Total (98) 00(00.00) 00(00.00) 98(100.00)

Total patients

M (73) 00(00.00) 00(00.00) 73(100.00)

F (52) 00(00.00) 00(00.00) 52(100.00)

Total M+F (125) 00(00.00) 00(00.00) 125(100.00)


89

MCV (Mean Corpuscular Volume)

The MCV count was shown in Table-20 and Figure-20. In all types of thalassemic patients

MCV count was very low inn male (98.63%); female (100.00%). Only one BTMi male

patient was observed with high MCV. None of the patients showed MCV in normal range.

Table-20. Showing prevalence percentage of MCV count in thalassemia patients.

Types of

Thalassemia

Sex

(Total patients)

MCV count (%)

Normal High Low

SCT

M (1) 00(00.00) 00(00.00) 1(100.00)

F (0) 00(00.00) 00(00.00) 00(00.00)

Total 00(00.00) 00(00.00) 01(100.00)

BTI

M (12) 00(00.00) 00(00.00) 12(100.00)

F (4) 00(00.00) 00(00.00) 04(100.00)

Total (16) 00(00.00) 00(00.00) 16(100.00)

BTMi

M (6) 00(00.00) 01(16.60) 05(83.33)

F (4) 00(00.00) 00(00.00) 04(100.00)

Total (10) 00(00.00) 01(10.00) 09(90.00)

TM

M (54) 00(00.00) 00(00.00) 54(100.00)

F (44) 00(00.00) 00(00.00) 44(100.00)

Total (98) 00(00.00) 00(00.00) 98(100.00)

Total patients

M (73) 00(00.00) 01(1.38) 72(98.63)

F (52) 00(00.00) 00(00.00) 52(100.00)

Total M+F (125) 00(00.00) 01(0.80) 124(99.20)


90

MCH (Mean cell hemoglobin)

The MCH was shown in Table-21 and Figure-21. In total the MCH count was normal in

majority patients. In nineteen patients MCH was observed below normal.

Table-21. Showing the percentage MCH count in thalassemia patients.

Types of

Thalassemia

Sex

(Total patients)

MCH (%)

Normal High Low

SCT

M (1) 01(100.00) 00(00.00) 00(00.00)

F (0) 00(00.00) 00(00.00) 00(00.00)

Total 01(100.00) 00(00.00) 00(00.00)

BTI

M (12) 09(75.00) 00(00.00) 03(25.00)

F (4) 04(100.00) 00(00.00) 00(00.00)

Total (16) 13(81.25) 00(00.00) 03(18.75)

BTMi

M (6) 06(100.00) 00(00.00) 00(00.00)

F (4) 04(100.00) 00(00.00) 00(00.00)

Total (10) 10(100.00) 00(00.00) 00(00.00)

TM

M (54) 45(83.33) 01(1.85) 08(14.81)

F (44) 36(81.81) 00(00.00) 08(18.18)

Total (98) 81(82.65) 01(1.02) 16(16.32)

Total patients

M (73) 61(83.56) 01(1.36) 11(15.06)

F (52) 44(84.61) 00(00.00) 08(15.38)

Total M+F (125) 105(84.00) 01(0.80) 19(7.20)


91

MCHC count

The MCHC was shown in Table-22 and Figure-22. In total 125 thalassemia patients MCHC

normal was: male (75.34%), female (73.07%) avarage (74.40%); high MCHC count: male

(17.80%), female (1.92%) total (19.20%); low MCHC count was male (6.84%), female

(5.76%) total (15.38%).

Table-22. Showing the MCHC count in thalassemia patients.

Types of

Thalassemia

Sex

(Total patients)

MCHC (%)

Normal High Low

SCT

M (1) 1(100.00) 00(00.00) 00(00.00)

F (0) 00 (00.00) 00(00.00) 00(00.00)

Total 01(100.00) 00(00.00) 00(00.00)

BTI

M (12) 11(91.66) 00(00.00) 01(8.33)

F (4) 04(100.00) 00(00.00) 00(00.00)

Total (16) 15(93.75) 00(00.00) 01(6.25)

BTMi

M (6) 06(100.00) 00(00.00) 00(00.00)

F (4) 04(100.00) 00(00.00) 00(00.00)

Total (10) 10(100.00) 00(00.00) 00(00.00)

TM

M (54) 49(90.75) 00(00.00) 05(9.25)

F (44) 37(84.09) 00(00.00) 07(15.90)

Total (98) 86(87.75) 00(00.00) 12(12.24)

Total patients

M (73) 67(91.78) 00(00.00) 6(8.21)

F (52) 45(86.53) 00(00.00) 07(13.46)

Total M+F (125) 112(89.0) 00(00.00) 13(10.04)


92

Platelets Count

The Platelet count was shown in Table-23 and Figure-23. In total 125 thalassemia patients

Platelets normal count was: male (75.34%), female (73.07%) total (74.40%); high Platelets

count: male (17.80%), female (1.92%) total (19.20%); low Platelets count was male (6.84%),

female (5.76%) total (15.38%). Overall the normal range platelet count is more than that of

the high range observed in twenty four patients and below range were eight in thalassemia

patients. The data corroborated with the results of Shivashankara et. al., (2008).

Table-23. Showing the prevalence percentage of platelet count in thalassemic patients.

Types of

Thalassemia

Sex

(Total patients)

Platelet count (%)

Normal High Low

SCT

M (1) 01(100.00) 00(00.00) 00(00.00)

F (0) 00(00.00) 00(00.00) 00(00.00)

Total 01(100.00) 00(00.00) 00(00.00)

BTI

M (12) 11(91.66) 00(00.00) 01(8.33)

F (4) 02(50.00) 02(50.00) 00(00.00)

Total (16) 13(81.25) 02(12.50) 01(6.20)

BTMi

M (6) 06(100.00) 00(00.00) 00(00.00)

F (4) 03(75.00) 00(00.00) 01(25.00)

Total (10) 09(90.00) 00(00.00) 01(10.00)

TM

M (54) 37(5.55) 13(24.07) 04(7.40)

F (44) 33(75.00) 09(20.45) 02(4.54)

Total (98) 70(71.42) 22(22.44) 06(6.12)

Total patients

M (73) 55(75.34) 13(17.80) 05(6.84)

F (52) 38(73.07) 11(1.92) 03(5.76)

Total M+F (125) 93(74.40) 24(19.20) 08(15.38)

Serum Ferritin Level:

The survey reports observed that the Serum ferritn levels are increased in all thalassemic

patients.


93

Blood Transfusion in thalassemic patients

Age of first blood transfusion

After diagnosis of thalassemia, blood transfusion was given to all patients. The survey reports

observed that the age of first blood transfusion varies.

Amount of blood transfusion

In thalassemic patients the blood was given on the basis of the patient’s age, weight and the

depending on the Hb percentage. Normally 200 ml blood was transfused at the time.

Frequency of blood transfusion

In thalassemic patients the frequency of blood transfusion were varies and depends upon the

Hb percentage. In some patient’s transfusion was given after two weeks and in some it was

given after three weeks or a month.

Pre and post blood transfusion Hb level.

The pre and post transfusion Hb level was shown Table-24 and Figure-24 a and 24b. Before

blood transfusion, low Hb level were observed in both patients.


94

Table-24. Showing the mean percentage of pre and post Hb and HCT count in thalassemia

patients.

Patient

Code

Patient

Initials Sex Diagnosis Pre Hb Post Hb Pre HCT Post HCT

1 BMH F TM 7.17 7.85 21.51 23.55

2 MAD M TM 6.33 7.75 18.99 23.25

3 MPA F TM 7.3 8.7 22.05 26.1

4 KPR F TM 6.98 8.15 20.94 24.45

5 JSS F TM 7.45 8.7 22.35 26.1

6 JP F TM 7.65 8.9 22.95 26.7

7 GDD F TM 7.2 8.4 21.6 25.2

8 DSS M BTI 6.79 7.9 20.37 23.7

9 KLB F TM 8.25 9.45 24.75 28.35

10 SGS M TM 7.17 8.95 21.51 26.85

11 MTM F TM 6.96 9.57 18.78 28.71

12 PST M TM 6.9 8.2 20.7 24.6

13 SSS M TM 8.05 9.3 24.15 27.9

14 KSS M TM 7.35 8.55 22.05 25.65

15 NA* F TM 7.3 8.5 21.9 25.5

16 MAR F TM 8.35 10.85 25.05 32.55

17 STS F TM 7.4 8.8 22.2 26.4

18 SSS M TM 5.67 6.87 17.02 20.62

19 CRR M TM 8.75 9.85 26.25 29.55

20 KSS M TM 6.5 8 19.5 24

21 GSA M TM 8.7 9.95 26.1 29.85

22 BSR F TM 7.5 8.75 22.5 26.5

23 CNT M BTMi 7.4 8.7 22.2 26.1

24 BKG F TM 6.8 8.6 20.4 25.8

25 SOS1 M TM 9.3 10.35 27.9 31.05

26 SOS2 M TM 8.5 9.75 25.5 29.25

27 SSS M TM 9.15 10.3 27.45 30.9

28 BLM M BTI 7.75 8.9 23.25 26.7

29 JPA F TM 8.35 9.6 25.05 28.8

30 TRM M TM 9.1 10.25 27.3 30.75

31 PYR M BTI 8.25 9.55 24.75 28.65

32 RGS* M BTI 8.5 9.65 25.5 28.95

33 MPP* F TM 8.3 9.5 24.9 28.5

34 GPO* M TM 8.15 9.25 24.45 27.75

35 TRI M TM 8.7 9.8 26.1 29.4


95


Patient

Code

Patient


36 KNS M TM 8.7 8.95 26.1 26.85

37 VDS M TM 8.9 9.9 26.7 29.7

38 PSB F BTMi 9.75 9.75 29.5 29.1

39 RSB M TM 9.01 10.3 27.03 30.9

40 IVY F TM 8.7 8.95 20.4 23.7

41 ARM M BTI 9.2 10.35 27.6 31.05

42 GAR M TM 9.55 10.75 28.65 32.25

43 KSM M TM 9 10.2 27 30.6

44 KAV F TM 9.2 10.3 2706 30.9

45 PUA F TM 8.5 9.6 25.5 28.8

46 DRS M TM 8.06 9.25 24.15 27.75

47 BMN M TM 7.25 8.4 21.75 25.2

48 LAB M TM 8.8 10.1 26.4 30.3

49 VDS M TM 8.9 9.2 26.7 27.6

50 SVP M BTMi 8.75 9.95 26.25 29.85

51 KAN F TM 8 9.25 24 27.75

52 SSS F TM 8.35 9.55 25.05 28.65

53 BUT M TM 7.4 8.15 22.2 24.45

54 MA M BTMi 7.9 9.3 23.7 27.9

55 RSS M BTMi 9.85 11 29.55 33

56 BNB M TM 7.7 8.85 23.1 26.55

57 IAS M TM 8.23 10.35 24.69 31.05

58 ISY M TM 7.85 9 23.55 27

59 TAS M BTI 9.45 10.7 28.35 32.1

60 PAM F TM 8.5 9.75 25.5 29.25

61 QSA M BTI 8.2 9.45 24.6 28.35

62 BSR M TM 6.75 8 20.25 24

63 RST F TM 7.1 8.4 21.3 25.2

64 QAS* F TM 9.7 10.8 29.1 32.4

65 GAM M TM 7.45 8.6 22.35 25.8

66 PHM M TM 8.85 10 26.55 30

67 GPS F TM 9.1 10.25 27.3 30.75

68 SV M TM 8.45 9.6 25.35 28.8

69 NDN F TM 8.6 9.75 25.8 29.25

70 MLD M TM 8.45 9.65 25.35 28.95


96


Patient

Code

Patient


71 PPV F TM 8.9 101 26.7 30.3

72 MVM M TM 8.95 10.2 26.85 30.6

73 KAS* M TM 8.85 10.05 26.55 30.15

74 CYS M SCT 7.65 8.9 22.95 26.7

75 LS M TM 6.85 8 20.55 24

76 GSS F TM 8.55 9.75 25.55 29.55

77 CGR* M TM 5.72 6.45 17.16 19.35

78 RS* M TM 5.2 6.45 15.6 19.3

79 JSS F TM 8.4 9.45 30 28.35

80 TNJ F TM 6.9 8 20.7 24

81 MPT F TM 8.15 9.3 24.45 27.9

82 BHH M TM 7.3 8.5 21.9 25.5

83 BSM M TM 9.5 10.8 28.5 32.4

84 NRB F TM 8.35 9.5 25.05 28.5

85 HNK M TM 8.8 9.3 26.4 27.9

86 KVM M BTI 8.5 9.75 25.5 29.25

87 NSA M TM 8.25 9.5 24.75 28.5

88 WAS M BTI 7.75 8.85 23.25 26.55

89 NAP M TM 8.3 9.5 24.9 28.5

90 MSS M TM 9.35 10.65 28.5 31.95

91 LSD M BTMi 7.85 9.1 23.55 27.3

92 ISS F BTMi 9 10.2 27 30.6

93 SAN F TM 7.1 8.3 21.3 24.9

94 GSM F TM 6.15 7.7 18.45 23.1

95 RKT F TM 6.2 8.25 18.6 24.75

96 RP* M TM 6.6 7.8 19.8 23.4

97 KSM M TM 8.2 9.4 24.6 28.2

98 MRR F TM 7.65 9 22.95 27

99 KPS F TM 8.65 8.7 28.54 26.1

100 MMM F TM 6.4 7.65 19.2 22.95

101 KBM F BTI 6.2 7.45 18.6 22.35

102 KJ* F TM 10 30 11.2 33.6

103 NPN F BTI 7.45 8.7 22.35 26.1

104 KAA F TM 7.95 9.15 23.85 27.45

105 PAD M TM 8.85 10.17 26.55 30.51


97


Patient

Code

Patient


106 HSD F TM 8.3 9.45 24.9 28.35

107 GAS M BTI 8.9 11.05 26.7 33.15

108 KSS M BTI 6.95 8.25 20.85 24.75

109 RYS M TM 8.1 9.35 24.3 27

110 LSD F BTMi 7.75 9 23.25 28.05

111 DML M BTMi 8.35 9.55 25.5 28.65

112 SS* M TM 8.1 9.4 24.3 28.2

113 PKM M TM 9.05 10.25 27.15 30.75

114 MGM M BTI 8.1 9.3 24.3 27.9

115 OBC F TM 7.9 9.1 23.7 23.4

116 PVD M TM 8.8 9.95 26.4 29.85

117 KKM F BTI 8.5 9.8 25.5 29.4

118 KR F TM 8.3 9.5 24.9 28.5

119 JVS M TM 7.4 9.65 22.2 28.95

120 TAN F TM 7.9 9.2 23.7 27.3

121 CLP F BTI 8.1 9.4 24.3 27.6

122 DAM M TM 6.6 7.7 19.8 23.1

123 RVS M TM 8 9.2 24 27.6

124 DRD F BTMi 6.5 7.8 19.5 23.4

125 JSS* F TM 8.6 9.75 25.8 29.25


98

Table-24a. Indicated the pre and post blood transfusion Hb range in thalassemia patients.

Types of

Thalassemia

Sex

(Total patients)

Pre Hb range (%)

Post Hb range (%)

Normal Low Normal Low

SCT

M (1) 00(00.00) 01(100.00) 00(00.00) 01(100.00)

F (0) 00(00.00) 00(00.00) 00(00.00) 00(00.00)

Total 00(00.00) 01(100.00) 00(00.00) 01(100.00)

BTI

M (12) 00(00.00) 12(100.00) 00(00.00) 12(100.00)

F (4) 00(00.00) 04(100.00) 00(00.00) 04(100.00)

Total (16) 00(00.00) 16(100.00) 00(00.00) 16(100.00)

BTMi

M (6) 00(00.00) 06(100.00) 00(00.00) 06(100.00)

F (4) 00(00.00) 04(100.00) 00(00.00) 04(100.00)

Total (10) 00(00.00) 10(100.00) 00(00.00) 10(100.00)

TM

M (54) 00(00.00) 54(100.00) 00(00.00) 54(100.00)

F (44) 00(00.00) 44(100.00) 00(00.00) 44(100.00)

Total (98) 00(00.00) 98(100.00) 00(00.00) 98(100.00)

Total

patients

M (73) 00(00.00) 73(100.00) 00(00.00) 73(100.00)

F (52) 00(00.00) 52(100.00) 00(00.00) 52(100.00)

Total M+F (125) 00(00.00) 125(100.00) 00(00.00) 125(100.00)


99

Pre and post blood transfusion HCT Count

The pre and post transfusion HCT count was shown in Table-24b and Figure-24 c and 24 d.

Before and after transfusion of blood, all thalassemic patients observed low HCT.

Table -24b. Showing pre and post blood transfusion HCT range in thalassemic patients

Types of

Thalassemia

Sex

(Total patients)

HCT Pre (%) HCT Post (%)

Normal Low Normal Low

SCT

M (1) 00(00.00) 01(100.00) 00(00.00) 01(100.00)

F (0) 00(00.00) 00(00.00) 00(00.00) 00(00.00)

Total 00(00.00) 01(100.00) 00(00.00) 01(100.00)

BTI

M (12) 00(00.00) 12(100.00) 00(00.00) 12(100.00)

F (4) 00(00.00) 04(100.00) 00(00.00) 04(100.00)

Total (16) 00(00.00) 16(100.00) 00(00.00) 16(100.00)

BTMi

M (6) 00(00.00) 06(100.00) 00(00.00) 06(100.00)

F (4) 00(00.00) 04(100.00) 00(00.00) 04(100.00)

Total (10) 00(00.00) 10(100.00) 00(00.00) 10(100.00)

TM

M (54) 00(00.00) 54(100.00) 00(00.00) 54(100.00)

F (44) 00(00.00) 44(100.00) 00(00.00) 44(100.00)

Total (98) 00(00.00) 98(100.00) 00(00.00) 98(100.00)

Total patients

M (73) 00(00.00) 73(100.00) 00(00.00) 73(100.00)

F (52) 00(00.00) 52(100.00) 00(00.00) 52(100.00)

Total M+F (125) 00(00.00) 125(100.00) 00(00.00) 125(100.00)

Malaria

The details of the screening of positive malaria patients are reported in Table-25.and Figure-

25. The prevalence was very less in thalassemic patients from Solapur District. Only one Beta

thalassemia intermedia male patient was observed Malaria positive out of 125 patients.

Transfusion-induced malaria may be a serious threat to the life in a group of special patients:

those who require many transfusions, those who have been treated with immunosuppressants,

and those who have undergone splenectomy. We observed transfusion-induced malaria in a

β-thalassemia major patient who had undergone splenectomy. Existing guidelines for

screening and excluding donors who have or have had malaria are inadequate, and serologic

screenings are too costly. We suggest some guidelines that may help prevent transfusion-


100

induced malaria, other diseases and complications. The present research data on malarial

infection in thalassemic patients from Solapur District can be corroborated with the reports of

Asian population study of Choudhury, et al. (1990). O'Donnell et al., (2009). This study also

got the less prevalence percentage of P. vivax.

Modiano et al. (1991) studied the protection against malaria morbidity in alpha-thalassemia

gene in a Nepalese population. Shear et al. (1998) in their studies they observed the

transgenic mice expressing human fetal globin are protected from malaria by a novel

mechanism. Roth et al. (1998) in their observation, malaria in beta-thalassemic mice and

their effects of the transgenic human beta-globin gene. The observations in this study were

that in SCT, BTI, BTMI and TM patients in Solapur District, were resistance to malaria due

to polytransfusion of blood.

Table-25. Prevalence of transfusion-induced malaria in thalassemic patients

Types of

Thalassemia

Sex (Total

patients)

Plasmodium

vivax (%)

P.

falciparum

(%)

P. malaria

(%)

P. ovale

(%)

SCT

M (1) 00 (00.00) 00 (00.00) 00 (00.00) 00 (00.00)

F (0) 00 (00.00) 00 (00.00) 00 (00.00) 00 (00.00)

Total (1) 00 (00.00) 00 (00.00) 00 (00.00) 00 (00.00)

BTI

M (12) 01 (08.33) 00 (00.00) 00 (00.00) 00 (00.00)

F (4) 00 (00.00) 00 (00.00) 00 (00.00) 00 (00.00)

Total (16) 01(6.25) 00 (00.00) 00 (00.00) 00 (00.00)

BTMi M (6) 00 (00.00) 00 (00.00) 00 (00.00) 00 (00.00)

F (4) 00 (00.00) 00 (00.00) 00 (00.00) 00 (00.00)

Total (10) 00 (00.00) 00 (00.00) 00 (00.00) 00 (00.00)

TM

M (54) 00 (00.00) 00 (00.00) 00 (00.00) 00 (00.00)

F (44) 00 (00.00) 00 (00.00) 00 (00.00) 00 (00.00)

Total (98) 00 (00.00) 00 (00.00) 00 (00.00) 00 (00.00)

Total patients

M (73) 01 (1.36) 00 (00.00) 00 (00.00) 00 (00.00)

F (52) 00 (00.00) 00 (00.00) 00 (00.00) 00 (00.00)

Total M+F

(125)

00 (00.80) 00 (00.00) 00 (00.00) 00 (00.00)


101

Transfusion –induced HCV, HIV, HBsAg and VDRL

The results of HCV, HIV, HBsAg and VDRL positive thalassemia patients showed in Table-

26; 27 and Figure-26. The HCV positive percentage was: male 8(10.95%), female 3(5.76%)

total 11(8.80%). The HIV positive and VDRL positive percentage was nil in all thalassemia

patients. The HBsAg positive percentage was: male 00(0.00%), female 2(5.76%) total

2(02.40%). The reports on tansfusion-induced pathoges are high frequency in splenectomized

patients, they acquired viruses such as HIV, syphilis, hepatitis B and C, iron overload and

defective adaptive/innate immune response (Smith et al., 1964; Piga and Monasterolo, 2001;

Ali et al., 2003;). Di Marco et al., (2008) reported that, in thalassemics, the severity of liver

damage (i.e. the finding of fibrosis and histological signs of cirrhosis) was clearly related to

persistent HCV infection. The reports observed and compared with study of Beeraj et. al.,

(2010) showed that the children suffering from beta-thalassemia major require repeated blood

transfusions which may be associated with dangers like iron overload and contraction of

infections such as HIV, HCV, and HBsAg which ultimately curtail their life span. On the

other hand, inadequate transfusions lead to severe anemia and general fatigue and debility.


102

Table-26. Prevalence of transfusion-induced malarial parasites, HCV, HIV, HBsAg, VDRL

and complications in the spleen and liver.

Patient

Code

Malarial

Parasites HCV HIV HBsAg VDRL Spleen Liver

1 Neg Neg NR Neg NR Normal Normal


3 Neg Neg NR Neg NR

Moderate

enlarge Normal











14 Neg Neg NR Neg NR

Moderate

enlarge Normal





Moderate

enlarge Normal


Mild

enlarge Normal













103


Patient

Code

Malarial






35 Neg Positive NR Neg NR Normal Normal


Moderate

enlarge Normal







Moderate

enlarge

Moderate

Enlarge



Moderate

enlarge

Moderate

enlarge

45 Neg Neg NR Neg NR Splenectomy

Moderate

enlarge



Moderate

enlarge

Moderate

enlarge


49 Neg Neg NR Neg NR Mild enlarge Normal




Moderate

enlarge Normal


Moderate

enlarge

Moderate

enlarge


Moderate

enlarge

Moderate

enlarge


Moderate

enlarge Normal


Moderate

enlarge

Moderate

enlarge


104


Patient

Code

Malarial




59 Neg Neg NR Neg NR Normal

Moderate

enlarge

60 Neg Neg NR Neg NR Normal

Moderate

enlarge



Moderate

enlarge

Moderate

enlarge


64 Neg Neg NR Neg NR Splenectomy Normal

65 Neg Positive NR Neg NR

Moderate

enlarge

Moderate

enlarge






Moderate

enlarge Normal

71 Neg Neg NR Neg NR Mild enlarge

Moderate

enlarge


Mild

enlarge


Moderate

enlarge Normal


Moderate

enlarge

Moderate

enlarge


105

Table-26. Continued...

Patient

Code

Malarial



Moderate

enlarge Normal


Moderate

enlarge


Moderate

enlarge Normal


Moderate

enlarge

Mild

enlarge



Mild

enlarge



Moderate

enlarge Normal




Moderate

enlarge Normal


87 Neg positive NR Neg NR

Moderate

enlarge Normal


Moderate

enlarge

Mild

enlarge




106


Patient

Code

Malarial





Highly

enlarge

Moderate

enlarge

94 Neg Neg NR Positive NR Mild enlarge

Mild

enlarge


Moderate

enlarge Normal


Moderate

enlarge

Moderate

enlarge


98 Neg Positive NR Neg NR Mild enlarge

Moderate

enlarge




Moderate

enlarge Normal


Moderate

enlarge

Moderate

enlarge

103 Positive Neg NR Neg NR

Moderate

enlarge

Moderate

enlarge


Moderate

enlarge

105 Neg Positive NR Neg NR Splenectomy Normal


107


Patient

Code

Malarial


106 Neg Positive NR Neg NR Splenectomy Normal


108 Positive Positive NR Neg NR Splenectomy

Mild

enlarge


Mild

enlarge

110 Neg Neg NR Positive NR Normal Normal


Moderate

enlarge

112 Positive positive NR Neg NR

Moderate

enlarge Normal



115 Positive Neg NR Neg NR Normal Normal




Moderate

enlarge

Mild

enlarge



121 Neg Positive NR Neg NR Splenectomy

Mild

enlarge


Moderate

enlarge

Moderate

enlarge



125 Neg Neg NR Positive NR Splenectomy Normal


108

Table-26a. Showing prevalence percentage of transfusion-induced pathogens like HCV,

HIV, HBsAg and VDRL.

Types of

Thalassemia

Sex

(Total patients)

HCV

Positive

(%)

HIV

Positive

(%)

HBsAg

Positive

(%)

VDRL

Positive

(%)

SCT

M (1) 00 (00.00) 00 (00.00) 00 (00.00) 00 (00.00)

F (0) 00 (00.00) 00 (00.00) 00 (00.00) 00 (00.00)

Total (1) 00 (00.00) 00 (00.00) 00 (00.00) 00 (00.00)

BTI

M (12) 02 (16.66) 00 (00.00) 00 (00.00) 00 (00.00)

F (4) 01 (25.00) 00 (00.00) 00 (00.00) 00 (00.00)

Total (16) 03 (18.75) 00 (00.00) 00 (00.00) 00 (00.00)

BTMi

M (6) 00 (00.00) 00 (00.00) 00 (00.00) 00 (00.00)

F (4) 00 (00.00) 00 (00.00) 01 (25.00) 00 (00.00)

Total (10) 00 (00.00) 00 (00.00) 01 (10.00) 00 (00.00)

TM

M (54) 06 (11.11) 00 (00.00) 00 (00.00) 00 (00.00)

F (44) 02 (04.54) 00 (00.00) 02 (04.54) 00 (00.00)

Total (98) 08 (8.16) 00 (00.00) 02 (2.04) 00 (00.00)

Total Positive

patients

M (73) 8 (10.95) 00 (00.00) 00 (00.00) 00 (00.00)

F (52) 3 (05.76) 00 (00.00) 03 (05.76) 00 (00.00)

Total M+F (125) 11 (8.80) 00 (00.00) 03 (02.40) 00 (00.00)

Liver

The liver of thalassemic patients was observed under three categories normal, mild

enlargement, moderate enlargement. Table-27 and Figure-27. Normal liver percentage was:

male 56(76.71%), female 38(73.07%) and in both male and female were 94(75.20%); mild

enlargement of liver: male 5(6.84%), female 4(7.69%) avarage 9(7.20%); moderate

enlargement of liver: male 12(16.43%), female 10(19.23%) avarage 22(17.60%). Liver

toxicity can occur as a direct consequence of iron toxicity and from transfusion-acquired

hepatitis. Liver function must be routinely screened in thalassemia patients on chronic

transfusion. The results compired with the observations of (Aach et al., 1991), due to blood

transfusions, many patients with β-thalassemia are infected with either hepatitis C virus (HCV) or

hepatitis B virus (HBV).


109

Hyperthyroidism

The hyperthyroidism was not found in any patients Table-27a. .

Alloimmunization

The alloimmunization (Table-27 and Figure-27a) was observed in male 1(1.36%), female

1(1.92%) avarage being 2(16.66%). This study includes red cell alloimmunization rate among

thalassemia patients on regular blood transfusion. Post-storage leukodepleted blood was used

for transfusion. It is not a standard practice in various centers to give phenotype-matched red

cells to patients with thalassemia. The use of phenotype matched blood is given only in

patients who have developed alloantibodies. Alloimmunization was an important finding in

patients with β-major-Thalassemia.

TM patients managed by regular transfusion regimen may develop anti-red cell

alloimmunization. If the alloantibodies are hemolyzing in nature, transfusion reaction may

occur, and provision of blood thereafter requires matching of the relevant blood group in

addition to `ABO" and Rh `D` matching. The hemolyzing nature of antibodies should be

determined in patients who develop these antibodies, and transfusion should be arranged

accordingly. Data regarding the impact of platelet refractoriness on morbidity and mortality

for thrombocytopenic patients are inconsistent. Failure to achieve platelet counts greater than

5 X 109/L significantly increases the probability of life-threatening bleeding. The rate of red

blood cell alloimmunization is relatively low in thalassemia patients. The age at first blood

transfusion and splenectomy were not statistically significant as risk factors for

alloimmunization in this study.

Patients were treated with immunosupressive drugs including prednisolone, intravenous

immunoglobulin, cyclosporine A and azathioprine and now all of them are in healthy

condition. Walker et al. (1989) observed the alloimmunization following blood transfusion.

Thompson et al. (2011) studied the Red cell alloimmunization in a diverse population of

transfused patients with thalassaemia. Iron level tests and serum ferritin tests were not done

time to time after and before blood transfusion by the patients.


110

Table-27. Showing the size of liver, hyperthyroidism and alloimmunization in thalassemic

patients.

Types of

Thalassemia

Sex

(Total

patients)

Liver Hyperthy

-roidism

Positive

Alloimmu-

nization

Positive

Normal Mild Moderate

SCT

M (1) 00 00 01 00 00

F (0) 00 00 00 00 00

Total (1) 00 00 01 00 00

BTI

M (12) 09 02 01 00 00

F (4) 02 01 01 00 00

Total (16) 11 03 02 00 00

BTMi

M (6) 04 00 02 00 00

F (4) 04 00 00 00 00

Total (10) 08 00 02 00 00

TM

M (54) 43 03 08 00 01

F (44) 32 03 09 00 01

Total (98) 75 06 17 00 02

Total

Positive

Patients (%)

M (73) 56

(76.71)

05

(06.84)

12 (16.43) 00

(00.00)

01

(01.36)

F (52) 38

(73.07)

04

(07.69)

10 (19.23) 00

(00.00)

01

(01.92)

Total M+F

(125)

94 (75.20) 09 (07.20) 22 (17.60) 00

(00.00)

02 (16.66)

Spleen and splenectomy

Table-28 and Figure-28 summarizes the spleen of thalassemic patients. It was observed

under five categories namely, normal, mild enlargement, moderate enlargement, highly

enlarged and splenectomy (removal of spleen). Normal size spleen percentage was: male

33(45.20%), female 28(53.84%) avarage 61(48.80%); Mild enlargement of spleen: male

9(12.32%), female 7(13.46%) avarage 16(12.80%); moderate enlargement of spleen: male

24(32.87%), female 8(15.38%) avarage 32(25.60%); highly enlargement of spleen: male

0(0.00%), female 1(1.92%) avarage 1(0.80%); splenectomy was: male7(9.58%), female

8(15.38%) avarage 15(12.00%). A highly enlarged spleen was observed in only one patient,


111

in sixteen patients spleen was mildly enlarge and in thirty two patients spleen was moderately

enlarge. In our series of 125 thalassemia patients, 15(12.00%) patients had undergone

splenectomy due to heavy iron overload by poly-transfusion. When the spleen continues to

enlarge, hypersplenism results and corrective measures such as splenectomy are required.

Spleen filters abnormal blood cells and removes them from the circulatory system; diseases

that result in abnormal red cells will cause the spleen to enlarge. Sickle cell disease,

thalassemia, and spherocytosis are examples of diseases that form unusually shaped cells that

cannot easily maneuver through the small blood vessels and capillaries of the body. If they

are not removed by the spleen, these abnormal cells can cause blood clots and decrease

circulation. However, removing them causes the spleen to swell and enlarge, the observations

are compired the reports of eMedicineHealth (2011). Splenomegaly is a common

complication caused mainly by massive erythropoieses the results corroborated with reports

of Cappellini et al., (2000a). Splenectomy is essential when spleen is highly enlarged. The

findings of Al-Salem et al. (1989); Smith et al. (1964); Sanefuji et al. (2006); Kheradpir et

al. (1985) and Phrommintikul et al. (2006) also corroborated with present data.


112

Table-28. Showing spleen enlargement in present study.

Types of

Thalassemia

Sex

(Total

patients)

Spleen

Normal Mild Moderate Highly

Enlarged

Spleenectomy

SCT

M (1) 00 00 01 00 00

F (0) 00 00 00 00 00

Total (1) 00 00 01 00 00

BTI

M (12) 09 01 01 00 01

F (4) 01 00 02 00 01

Total (16) 10 01 03 00 02

BTMi

M (6) 02 02 02 00 00

F (4) 02 01 00 00 01

Total (10) 04 03 02 00 01

TM

M (54) 22 06 20 00 06

F (44) 25 06 06 01 06

Total (98) 47 12 26 01 12

Total Positive

Patients (%)

M (73) 33

(45.20)

09

(12.32)

24 (32.87) 00

(00.00)

07

(09.58)

F (52) 28

(53.84)

07

(13.46)

08 (15.38) 01

(01.92)

08

(15.38)

Total

M+F

(125)

61

(48.8)

16

(12.80)

32 (25.60) 01

(0.80)

15

(12.00)

Medication

As documented in Table-29 and Figure-29, the thalassemic patients mediacation was: Anti

TB; Desirox-500mg male (0.00%), female (1.92%) avarage (0.8%); Asunra 400mg male

(1.36%), female (1.92%) avarage (16.0%); Aurvedic male (2.73%), female (0.00%) avarage

(16.0%); Calcium-X; Kelfer male (0.00%), female (1.92%) avarage (0.8%); Calpol; Bevon

male (1.36%), female (0.00%) avarage (0.8%); Dyton; Folvite male (1.36%), female (0.00%)

total (0.8%); Folvite male (6.84%), female (25.0%) avarage (14.4%); Insulin male (1.36%),

female (0.00%) total (0.8%); Kelfer male (17.80%), female (7.69%) avarage (13.6%); Kelfer;

Folvite male (0.00%), female (1.92%) avarage (0.8%); Loser male (1.36%), female (0.00%)


113

total (0.8%); no medication male (54.73%), female (59.61%) avarage (56.8%). Total fifty four

patients were gives treatment with different drugs by suggestions of the medical experts, the drugs

are namely anti TB drugs, Desirox-500mg, Asunra, ayurvedic treatment, Calcium-X; Kelfer,

Calpol, Bevo, Dyton, Folvite, Insulin.

Table-29. Showing the medication given for thalassemic patients.

Sex Medication Male (%) Female (%) Total Male +

Female (%)

Medication

Anti TB; Desirox-500mg 0 (0.00) 1 (1.92) 1 (0.8)

Asunra 400mg 1 (1.36) 1 (1.92) 2 (16.0)

Aurvedic 2 (2.73) 0 (0.00) 2 (16.0)

Calcium-X; Kelfer 0 (0.00) 1 (1.92) 1 (0.8)

Calpol; Bevon 1 (1.36) 0 (0.00) 1 (0.8)

Dyton; Folvite 1 (1.36) 0 (0.00) 1 (0.8)

Folvite 5 (6.84) 13 (25.0) 18 (14.4)

Insulin 1 (1.36) 0 (0.00) 1 (0.8)

Kelfer 13 (17.80) 4 (7.69) 17 (13.6)

Kelfer; Folvite 0 (0.00) 1 (1.92) 1 (0.8)

Loser 1 (1.36) 0 (0.00) 1 (0.8)

No 40 (54.73) 31 (59.61) 71 (56.8)

Mortality

The mortality rates of study patients were also examined during the study period (Table-30

and Figure-30) showing probably age specific mortality. In 6-10 years the mortality rate was:

male 5(6.84%); female (0.00%); avarage 5(4%). The mortality rate was: male 3(3.10%);

female 5(9.61%); avarage 8(6.4%) in age between 11-15 Years and 16-18 years: male

2(2.73%); female 3(5.76%); avarage 5(4%). This leads to increased mortality during 11-15

years of age. As a result, transfusional iron overload can cause increased morbidity and premature

mortality in thalassemia patients. The results compared with the findings of the Olivieri and

Brittenham (1997) and Darbari et al. (2006). The greater the transfusion requirement, the greater the

risk of premature mortality was observed during the study. The only way to prevent the disease

and reduce the morbidity and mortality is by educating the patients and parents. For this

reason in this present study, awareness among parents of thalassemic patients regarding the

disease was evaluated. Epidemiological transition caused by improvements in hygiene,


114

nutrition, proper medication, blood transfusions and control of infection that has reduced

childhood mortality.

Table-30. Showing the prevalence percentage of mortality in thalassemic patients.

Types of

Thalassemia

Sex

(Total patients)

Mortality

6 Months

to 5 Years

6 Years -

10 Years

11 Years- 15

Years

16 Years to

18 Years

SCT

M (1) 00 00 00 00

F (0) 00 00 00 00

Total (1) 00 00 00 00

BTI

M (12) 00 00 00 01

F (4) 00 00 02 00

Total (16) 00 00 02 01

BTMi M (6) 00 01 00 00

F (4) 00 00 00 00

Total (10) 00 01 00 00

TM

M (54) 0 04 03 01

F (44) 00 00 03 03

Total (98) 00 04 06 04

Total patients

(%)

M (73) 00 (0.00) 05 (6.84) 03(4.10) 02 (2.73)

F (52) 00 (0.00) 00 (0.00) 05 (9.61) 03 (5.76)

Total M+F (125) 00 (0.00) 05(4.0) 08 (6.4) 05 (4.00)


115

6. SIGNIFICANCE OF THE STUDY

The results showed that the high prevalence of thalassemia was observed in the Solapur

District.

Screening of children, pregnant women, and individuals visiting public health facilities is

effective in identifying individuals at risk who require further testing. A simple CBC count,

with emphasis on the RBC counts and indices, including the mean corpuscular volume

(MCV), mean corpuscular Hb (MCH), and RBC distribution width (RDW), is the main

component of such screening processes. Persons suspected to be positive for thalassemia are

checked for elevated levels of Hb for confirmation. In some situations, this simple method

was not adequate, and further testing, including analyses of globin chain synthesis, must be

performed to reach a final diagnosis.

To safeguard the quality of blood transfusion services , the recommendations of WHO and

other agencies should be taken into considerations.

At the time of study, some patients were unaware of their diabetes status. The patients did not

know that their blood was infected by pathogenic organisms. The thesis data was also helpful

for all thalassemic patients in Solapur District and in Country to plan carrier screening,

counseling, awareness of the diseases, prenatal diagnosis and related services. The

information provided here was very useful for efficient evaluation of novel treatment

methods, educational, informational purposes and management strategies of potential benefit

for children and adults with thalassemia.

Further devotement of new methodology, research plans and directs research and research

training and career development programs, on the causes, prevention, and treatment of blood

diseases, including anemias, sickle cell disease, and thalassemia.

Present research work was helpful for extending knowledge of the thalassemia, its prevention

and treatment. It may also prove useful for policy makers, health professionals, patients and

parents, and the community at large.


116

It will also proved helpful for screening the right of every patient for equal access to quality

medical care, and the safety and adequacy of blood. This research work is intended for a wide

audience with the expectation that national health authorities, policy-makers, scholars,

researchers, health workers and others committed to the advancement of public health in the

Asian region will make good use of it.


117

7. CONCLUSION

Over 125 patients affected by thalassemia live in Solapur District, Maharashtra, India.

Thalassemia is a blood disease and is common in both sexes. Thalassemia was suspected in

all these patients with age six months to eighteen years, the prevalence percentage of TM was

more than the BTI, BTMi and SCT. The prevalence percentage of thalassemia in specific

ethnic groups. In Solapur District the high frequency of thalassemic patients were observed in

the area of Solapur City, Pandharpur Taluka and in South Solapur as compared to other

Talukas.

In our study, only one SCT patient was found, total BTI patients were sixteen, BTMi were

four and TM were ninety eight. Hence the prevalence percentage of TM were very high in

Solapur District as compared to SCT, BTI and BTMi.

Patients present with pallor, poor appetite, poor growth, lethargy and irritability was

observed. Only one mentally challenged female patient was observed. As thalassemia has an

impact on the different psychosocial life aspects, it is very essential to provide psychosocial

support, including progress of the disease in the patients and parents for clear understanding.

In the present study, the age of thalassemia patients was in between the range of 6 months to

18 years. The mean age was around 8.75 years. The numbers of patients in the age group 6

months to 3 years are more. There were 19 Male and 15 female with t avarage 34(27.2%)

patients in 6 months-3 years age group. Patients between 4-6 years, 12 Male and 6 female

with avarage 18(14.4%); patients in 7-10 years, Male 22, female 11, avarage 33(26.4%); in

11-13 years Male 9 female 11 total 20(16%); in between 14-16 age Male7, female 3, avarage

10(8%); in 17-18 years Male 4, female 6, avarage 10(8%), Maximum patients was:

34(27.2%) were in the age group of 6 months 3 years and minimum patients were : 10(8%) in

both age group of 14-16 and 17-18 years age.

The present study comprised of males and females. A higher incidence of thalassemia was

observed in male than female. Thalassemia was the serious medical problem. In the present

research work, the findings are the failure to thrive after six months of age, stunting in height

and weight. It indicates majority thalassemic patients showed poor growth in poly-transfused

beta thalassemia patients. The observations showed the consanguinity rate was very high


118

(>49%) in male and female. That indicates the need for implementing a comprehensive

genetic preventive program for the eradication of thalassemia in Solapur District.

As per the pedigree analysis of the patients observed, most of the parents were thalassemia

minor. The majority (>22.4%) of thalassemia patients were illiterate with only 1.6% with a

higher education. Only one case was reported under psychological burden. It is evident from

the findings of our study that besides the clinical burden, thalassemic have to shoulder very

low psychosocial impact associated with the disease. There was no any correlation between

the psychological impact and the gender. Furthermore, the concept of non-directive genetic

counseling is incompatible with a campaign against consanguineous marriage. The

prevalence percentage was very high in Hindu and muslin as compared in Jain and Sindhi

community. In the present study the non vegetarian thalassemic patients are more than the

vegetarian. Rodent face and saddle nose are more in thalassemic patients as compared to

maxillary protrusion, maxillary anterior teeth spacing and anterior open bite. Only one case

was observed in mucosal discoloration. There was not any relationship between the

complications and sex but skull and face deformities can be closely related to the patient's

age.

In conclusion the hematological parameters vary as compared to normal ranges. The normal

WBC was 80.80% and below normal range percentage of WBC was observed nil in all

patients as compared to high WBC count (>19%). RBC count above range was nil in both

sexes. But in TM patients RBC count below normal was observed in majority patients

(>95%). RBC indices showed microcytic anemia. TM is characterized by reduced Hb level

(<7 g/dl). Hematological investigations revealed the low Hb levels <8.0 g/dL (falling further

with increasing age and severity) with severe microcytosis and hypochromia and increase

target cells in the peripheral film.

All thalassemic patients’ hemoglobin, HCT count was very low, not a single patient was

observed in normal and with high range. MCV count was very low (>99%) in majority

patients. The MCH and MCHC readings was normal in majority patients but nineteen

patients MCH and thirteen patients MCHC was observed below range. Overall the normal

range platelet count is more than that of the high range observed in twenty four patients and

below range was eight in thalassemia patients.


119

The pre and post transfusion normal hemoglobin and HCT count was nil in all patients but

below range was observed in all patients, which is comparable to studies conducted by other

authors. Electrophoresis is a gold standard for the diagnosis of thalassemia but it is not

available at all places. Thus several attempts have been made to diagnose the condition by

using red cell indices.

Only the history of one thalassemic patient was showed Plasmodium vivax infection due to

blood transfusion. Recently available kits are useful for testing the positive malarial cases and

it helps to prevent transfusion-induced malaria, other diseases and other complications. This

extensive and well-executed study concludes that SCT, BTI, BTMi, and TM protect

individuals against acquiring malarial infection, They become symptomatic but develop very

low parasite densities. However, the study also concludes that the risks of developing severe

malaria, especially malarial anaemia, are reduced in people with SCT,BTI, BTMi and TM

patients and they reduced risk of malaria-specific mortality.

The HIV positive and VDRL positive percentage was nil in all thalassemia patients. The

HBsAg positive observed 2.40%. The HCV positive were observed 8.80% due to blood

transfusion.

The mild liver enlargement was observed in nine patients and moderate enlarge liver was

observed in twenty two patients. Liver function and hepatitis serology must be routinely

screened in thalassemia patients on chronic transfusion.

The hyperthyroidism was not seen in any thalassemic patients, but the alloimmunization was

observed in one male and one female. A highly enlarged spleen was observed in only one

patient, in sixteen patients spleen was mild enlarge and in thirty two patients spleen was

moderately enlarge. Total fifteen patients were done splenectomy due to heavy iron overload

by poly-transfusion. Total fifty four patients were given treatment with different drugs by

suggestions of the medical expert, the drugs are namely anti TB drugs, Desirox-500mg,

Asunra, ayurvedic treatment, Calcium-X; Kelfer, Calpol, Bevo, Dyton, Folvite, Insulin,

Loser whichever necessary. For supportive treatment mainly by blood transfusion and

medications is freely available in various blood banks at Solapur.


120

The age group of 6-10 years the mortality rate in male were 6.84%; female 0.00%, in the age

between 11-15 Years and 16-18 years: male 2.73%, female 5.76%, avarage 4%. This leads to

increased mortality during 11-15 years of age. As a result, transfusion iron overload can

cause increased morbidity and premature mortality in thalassemia patients.

In conclusion, blood transfusion therapy and optimal safety of transfused blood are the key

concepts in the protocol of treatment of thalassemia. The maintenance of red cell viability

and function during storage, to ensure sufficient transport of oxygen, maintainance of

appropriate hemoglobin level was essential. Avoidance of adverse reactions, including

transmission of infectious agents like malarial parasites, other pathogenic protozoans, HCV,

HIV, HBsAg, VDRL, microorganisms and other complications are essential.

Intake of Milk, a rich source of calcium, should be at least 500 ml daily, particularly because

it helps to prevent osteoporosis. In Thalassemia, although most of the iron overload is due to

blood transfusions, a small amount of iron is also absorbed from the diet. The absorption of

iron is higher when the hemoglobin is low. Iron chelators such as Desferal and Deferiprone

not only bind iron but also some Zinc and excrete it in the urine. The main nutritional sources

of Zinc are animal foods (meat and dairy products) and wholemeal cereals. In thalassemia,

because of the excess iron in the body, there is a higher risk of oxidative damage. The main

antioxidants are vitamin E, vitamin C, carotenoids and flavonoids are to be given to patients.

Knowing the results of thalassemia screening can impact decisions for prenatal diagnosis

before marriage and well before pregnancy. In conclusion this thesis offers various types of

data that may be useful in management and prevention of thalassemia in India and abroad.

Future research directions

The goal for future research in thalassemia is not only to introduce new strategies of

diagnosis and treatment of thalassemia but also to discover ways to prevent transfusion-

induced malaria, HCV, HIV, HBsAg, VDRL and other complications in thalassemic patients.

Current work deals with specific complications in thalassemia patients such as orofacial

complications, consigunity, ethnicity, hyperthyroidism, Alloimmunization, Hb level at pre

and post blood transfusions, anthropologic measurements, hematological study and

transfusion-induced or transmitted pathogens, complications in liver, spleen,

alloimmunization etc. Efforts should be towards promoting awareness of thalassemia


121

amongst the public and health professionals, and promoting the establishment and

improvement of policies aimed at the prevention and medical treatment of thalassemia.


122

LIST OF ABBREVIATIONS

Symbol Abbreviations

α Alpha

HBA Adult Hemoglobin

β Beta

BT Beta thalassemia

TM Beta-Thalassemia Major

BTMi Beta Thalassemia Minor

BTI Beta Thalassemia Intermedia

CBC Complete Blood Count

0C Degree Celsius

δ Delta

ε Epsilon

F Female

γ Gamma

gm Gram

HCT Hematocrite

HgB Hemoglobin

HBA2 Hemoglobin A2

HbF hemoglobin F (Fetal hemoglobin)

HbS hemoglobin S (Sickle hemoglobin)

HBsAg Hepatitis B Surface Antigen

HCV Hepatitis C Virus

HIV Human Immunodeficiency Virus

Kg Kilogram

M Male

M.C.V. Mean Cell Volume

M.C.H. Mean Cell Hemoglobin

M.C.H.C. Mean Cell Hemoglobin concentration

µ micro

µg micro-gram

µl Micro-Liter

ml Milliliter

Neg Negative

NR Non Reactive

NS Not seen

% Percentage

R.B.C. Red blood cells

SCT Sickle cell thalassemia

θ Theta

VDRL Venereal Disease Research Laboratory

W.B.C. White blood Cells

ζ Zeta


123

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………………………………………………………………………………………………..


147

ANNEXES

Aproval letter as a Technical Supervisor (Component Section) at blood bank.

Food and Drug Administration, Government of Maharashtra, Pune, approved by me as a

Technical Supervisor (Component Section) at blood bank last 10 Years. Letter Reference No.

Med/Prod/805/07/2 Dated 12th March 2007.

Thalassemic Patients Data Bank

Thalassemic patients’ data sheet, photography, consent forms, all reports and peripheral

blood smear slides were deposited at Protozoology Laboratory, Dr. Babasaheb Ambedkar

Marathwada University, Aurangabad, Maharashtra, India.


148

LIST OF PUBLICATIONS

1. Patil S.S., Nikam S.A., Dama S.B., Chondekar R.P., Kirdak R.V. and Dama L.B.

(2011). Prevalence of hepatitis-B surface antigen (HBsAg) positivity in Solapur District,

Maharashtra State, India. Bangladesh Journal of Medical Science. 10(2): 91-94. (ISSN:

1607-0755 EISSN: 2076- 0299).

2. Nikam S.V., Dama S.B., Saraf S.A., Jawale C.S., Kirdak R.V., Chondekar R.P.,

Patil S. S., Deshmukh P.S., Shaikh F.I., and Dama L.B. (2011). Prevalence of red cell

alloimmunization in repeatedly transfused patients with Β- Thalassemia in Solapur

District, Maharashtra State, India. UGC-Sponsored Proceedings of the National-Level

Workshop cum Seminar on ‘Bio-Resources for Bio-Industries and Economic Zoology’,

January- 2011, Solapur, Maharashtra, India. 1-3. (ISBN 81-903603-6-1).

3. Wath M. R., Chondekar R. P., Saraf S. A And Dama S. (2011). Antimicrobial

Evaluation of Three Species Of Cassia Siamea L. UGC-Sponsored Proceedings of the

National-Level Workshop cum Seminar on ‘Bio-Resources for Bio-Industries and

Economic Zoology’, January- 2011, Solapur, Maharashtra, India. 4-6. (ISBN 81-903603-

6-1).

4. Mohd Iliyas., Shaikh, F. I., Quazi Salim., Sayri Abdula and Dama, S. B. (2011).

Behavioral assessment of heavy metal on freshwater crab Barytelphusa cunicularis.

UGC-Sponsored Proceedings of the National-Level Workshop cum Seminar on ‘Bio-

Resources for Bio-Industries and Economic Zoology. 52-54, (ISBN 81-903603-6-1).

5. Tembhurkar V.R., Chintawar S.S., Dama S.B., Arvikar P.G. and Dama L.B. (2012).

Detection of respiration deficiency mutants of Saccharomyces cerevisiae by MBRT test.

DAV International Journal of Science. 1(1): 29-31. ISSN: 2277-5536 (Print); 2277-5641

(Online).

6. Nikam S.V., Dama S.B., Jawale C.S., Deshmukh P.S., Patil S.S. , Tembhurkar V.R.

and Dama L.B. (2012). Height and weight correlation in thalassemic patients from

Solapur District, Maharashtra, India. DAV International Journal of Science. 1(1): 54-57.

ISSN: 2277-5536 (Print); 2277-5641 (Online).


149

Figure -1. Global distribution of thalassemia, hemoglobins S, C, D and E.

Figure-2. A- Map of India


150

B-Map of Maharashtra

C- Map of Solapur Solapur District.


151

Figure-3. Showing the Talukawise geographical distribution and prevalence percentage of

the thalassemic patients in Solapur District.

Figure -4. Showed type of thalassemic patients entering the study (n=125)

Figure-4a. Showing prevalence percentage of different types of thalassemia in Solapur

District age between 6 months to 18 years.


152

Figure -5. Showing the mean height (Inch) and weight (Kg) in thalassemia patients.

Figure- 6. Showing prevalence percentage of growth in thalassemic patients.


153

Figure-8. Showing the education in thalassemic patient.

Figure-9. Showing the prevalence percentage of the skin color in thalassemia patients


154

Figure- 10. Showing the prevalence percentage of hearing, visual impairement and

psychological complications in thalassemia patients.

Figure-11. Showing prevalence percentage of the consanguineous marriages in the parents of

the thalassemic patient.

Figure 12. Showing the prevalence percentage of ethnicity in thalassemic patients.


155

Figure-13. Showing prevalence percentage of thalassemic patients’ diet.


156

Figure-14. Prevalence of orofacial complications in thalassemia patients: Rodent face.

Figure -14a. Prevalence of orofacial complications in thalassemia patients: Saddle nose.

Figure-14b. Prevalence of orofacial complications in thalassemia patients: Maxillary

protrubution.


157

Figure-14c. Prevalence of orofacial complications in thalassemia patients: Maxillary anterior

teeth spacing.

Figure -14d. Prevalence of orofacial complications in thalassemia patients: Anterior open

bite

Figure-14e. Prevalence of orofacial complications in thalassemia patients: Deep bite.


158

Figure-14f. Prevalence of orofacial complications in thalassemia patients: Mucosal

discoloration.

Figure- 15.


159

Figure-15. Photomicrograph of a blood film. Sickle cell thalassemia. (Black arrow

indicates: target cells; Green arrow indicates: distorted tear drop cells; Blue arrow:

hypochromic microlytic cells).

Figure15a. Photomicrograph of a blood film. β-thalassemia Intermedia. (Black arrow

indicates: target cells; Green arrow: distorted tear drop cells; Brown arrow: echinocytes;

Blue arrow: hypochromic microlytic cells).


160

Figure- 15. Continued...

Figure-15c. Photomicrograph of a blood film. β-thalassemia minor. (Green arrow

indicates: hypochromasia, anisochromasia and anisocytosis).


161

Figure-15d. Photomicrograph of a blood film. Thalassemia Major. Shows

hypochromasia, anisochromasia and anisocytosis and numerous poikliocytes and cell

fragments. (Black arrow indicates: target cells; Green arrow indicates: hypochromasia,

anisochromasia and anisocytosis; Red arrow: envelope form cell)

Figure- 15. Continued..

Figure -15e. .Photomicrograph of a blood film. Thalassemia Major, after splenectomy.

Shows many target cells grossly deficient in haemoglobin. The relatively dark staining

target cells are normal cells, which have been transfused. (Black arrow indicates: target

cells)


162

Figure-16. Showing the prevalence percentage of WBC count in thalassemia patients.

Figure-17. Showing prevalence percentage of RBC count in thalassemic patients.


163

Figure-18. Showing the prevalence percentage of Hb in thalassemic patients.


164

Figure-19. Showing the prevalence percentage of HCT count in thalassemia patients.

Figure-20. Showing prevalence percentage of MCV count in thalassemia patients.

Figure-21. Showing the prevalence percentage MCH count in thalassemia patients.


165

Figure-22. Showing the range of MCHC in thalassemic patients.


166

Figure-23. Showing the prevalence percentage of platelet count in thalassemic patients.

Figure-24a. Showing the range of pre transfusion Hb in thalassemic patients.

Figure-24b. Showing the range of post transfusion Hb in thalassemic patients


167

Figure-24c. Showing the range of pre transfusion HCT in thalassemic patients.

Figure-24d. Showing the range of post transfusion HCT in thalassemic patients


168

Figure-25. Showing prevalence Percentage of malaria history in thalassemic patients.


169

Figure-26. Showing prevalane percentage of HCV, HIV, HBsAg and VDRL in thalassemia

patients

Figure-27. Showing prevalence percentage of liver dysfunction in thalassemia patients

Figure-27a. Showing prevalence percentage of hyperthyroidism and alloimmunization in

thalassemia patients


170

Figure-28. Showing prevalence percentage of spleen and splenectomized thalassemic

patients

Figure-29. Showing the medication in thalassemia patients.


171

Figure-30. Showed the mortality rates of thalassemic patients during the study period.