acute lymphoblstic leukemia

By:Dr. Muhammad Wasiq ParachaHouse Officer,Paediatrics Unit 2Civil Hospital Karachi

Case Presentation

A 9 Years old Aiman, female child, weighing 27 kg, vaccinated, resident of Surjani Town – Karachi, came to ER with complain of

Fever for 2 weeks

Pallor for 1 week

Bleeding from gum for 5 days

History

History of Presenting Complain :

According to child’s mother child was in his usual state of health 2 weeks back when she developed fever high grade ,documented up to 102’F associated with rigors and chills, fever temporarily relieved by antipyretics

Mother also noticed pallorness since 1 week which increased gradually

There is history of fresh bleeding from gum near about 3 tablespoon in quantity which was stopped after I/V injections.

Took multiple antibiotic but no documentation available

There is no hx of bruises, melana, hematmesis, epistaxsis, fits, loose motion, constipation, anorexia,

Hx of hospitalization 1 wk back due to similar complaints at NICH where they made diagnosis of Enteric with complication but they got lama from there, then they went to private hospital where CBC was done which showed pancytopenia and they started tab deltacortil

PCV and Platelets were also transfused 1 week back at private hospital

Systemic review:

GIT: no diarrhea, abdominal distention or oral ulcers. CVS: no difficulty breathing on walking. Respiratory System: no chronic cough or recurrent chest

infections. CNS: No seizures, drowsiness, headache or black outs Urinary: no hematuria or burning micturation. Endo: no palpitations, heat intolerance, joint pain,

photosensitivity Msk: bodyaches

Past history:

Past medical history: none

Past surgical history: none

Transfusion: none

Allergies: none

Drug history: none

Birth history

Antenatal – Booked Case, No H/O Any Rash, GDM, PIH. No H/O Drug Intake.

Natal - NVD. Uncomplicated birth.

Post Natal - Cried immediately after birth, No H/O NNJ or NICU Admission. Meconium passed within first 24 hrs.

Nutrition

Breast fed for 6 months only, then weaning started and mother’s milk tapered off. Weaning consisted of cerelac, dalia, banana.

Breakfast: 1 cup tea + 1 paratha (egg approx 2 times aweek)Lunch: ½ - 1 rotis with salan cooked at home, which isusually vegetables or pulses. Meat is also cooked once perweek Dinner: same as aboveSnacks: tea in evening.Average intake of fruits. Taking 60-70% of required calories.

Developmentally Appropriate for age .

Vaccinated according to EPI. Card not available. BCG scar present.

Personal history

Apetite: decreased during illness.

Bowel: normal.

Micturation: normal

Weight loss: Undocumented

Family history

17 yrs 15 yrs 12 yrs 9 yrs 6 yrs

40 yrs 38 yrs

3yrs

4th product of consanguineous marriageParents and sibling are alive and healthy.No history of recurrent transfusions in family, either in parents, siblings or relatives.No h/o TB, asthma or any other chronic illness in family.

Socioeconomic history:

Father is a shopkeeper. Mother is house wife. They live in own house of 3 rooms, 8 people living in it. Use unboiled water for drinking. Good exposure to sunlight or air. Belong to lower middle class.

General Physical Exam:

Alert, oriented, cooperative child, lying on bed comfortably. Heart rate: 102/minResp. rate: 30/minTemp.: 98 FBP: 100/60 mm Hg (50th centile)SPO2: 98% on room air

Anthropometry

OFC: 53cmHeight: 145cm (at 50th percentile)Weight: 27 kg (at 50th percentile)

Anemia= ++ (moderate)Cyanosis= absentJaundice= absentClubbing= absentKoilonychia= absentDehydration= absentAnkle Edema = absentNo lymphadenopathyThyroid= Not palpable

Petachae on both lower limbs

CVS

On inspection, no bulge or visible pulsations.On palpation, no thrill. Apex beat located in 4th ICS, at mid clavicular line, not heaving. On auscultation: heart sounds normalPulses palpable in all four limbs, good volume. No radio femoral delay. Capillary refill <2 sec.

Respiratory System

On inspection, symmetrical shape and movement of chest.On palpation, trachea is central.Percussion is resonant all over equally. On auscultation, Normal vesicular breathing B/L

Abdominal Examination:

On inspection, abdomen is full, moving symmetrically with respiration.

On palpation, soft, non-tender. Liver palpable 2cm bcm. Liver span 9 cm. Spleen also palpable 2 cm

On percussion, abdomen is tympanic, no shifting dullness or fluid thrill found.

On auscultation, gut sounds are audible.

Genito-urinary Examination:Normal

CNS Exam

GCS was 15/15.Higher mental functions: intact.Speech: fluent, appropiate for age.Motor examination: Bulk symmetrical in all four limbs. Tone,

power, reflexes normal. Planters down going bilaterally.Sensory system: Intact.Cranial nerves: Intact.Cerebellar signs: Negative.SOMI: Negative.Pupils: BERL.

A 9 yrs old female weighing 27kg , vaccinated came with c/o

Fever for 2 weeksPallor for 1 week Bleeding from gums: for 5 days

Petechiae on lower limb for 1 dayHx of transfusion of PCV and platelets 1 week back

O/E : pale looking child, vitally stable Systemic exam: liver n spleen palpable 2 cm

Case Summary

Differential Diagnosis?

1. Enteric with complication

2. Malaria

3. Malignancy

4. Dengue fever

Differential Diagnosis

• Orally allowed• TPR• Inj Paracetamol 27 cc I/V sos• Inj Ceftraixone 1gm I/V BD• Inj Artisunate 65mg I/V stat• Inj Omeperazole 27mg I/V OD • PCV + Plt transfused• Recommend admission

Management in ER

DATE 22/11 24/11 28/11

HB 7.9g/dl 9.9g/dl 8.6g/dl

MCV 85.6 83.3 84.6

MCH 27.8 27.6 28.1

MCHC 32.5 33.1

TLC 4.4 3.6 2.2

N% 23 22 33

L% 76 75 64

Plt 15 13 25

NORMAL VALUE

13-14.5

80-90

31-34

41-47

4.5-13.5

58

35

150-350 x 10^3

Investigations

DATE 22/11 28/11

BUN 8 5

Cr 0.30 0.50

Na 144 137

K 4.6 4.2

Cl 101 97

Ca 9.0 9.5

CRP 12.9 58.4

Malaria parasite: NegativeMP-ICT: NegativeDengue Serology: NegativeBlood C/S: No bacterial Growth

Blood Morpholgy

Hypochromic, Anisocytosis, Roulex (+), Platelet Low.Advice Bone Marrow Aspiration D/R

Bone Marrow Aspiration

There is total replacement of all haemopoietic series cells by sheet of blast cells, most of these cells are uniform in size and shape with scanty cytoplasm and indistinct nucleoli; lymphoblastic.

Rest of the haemopoietic series cells are markedly depressed

Conclusion: peripheral blood and bone marrow examination finding are in consistent with ACUTE LYMPHOBLASTIC LEUKEMIA, most likely ALL-1(according to FAB morphological classification)

Acute Lymphoblastic Leukemia.Diagnosis

Leukemia is the most frequent childhood malignancy, approx. 30% of all childhood cancers.

Incidence is highest in children 2 to 5 years of age, and more in boys.

Both genetic and environmental factors may contribute to its development.

Introduction

A malignant progressive disease in which the bonemarrow and other blood forming organs produce increase no of immature or abnormal leukocytes.

They supress production of normal blood cells leading to anemia and other symptons

Definition

LEUKEMIAS

CHRONIC LEUKEMIA

LYMPHOID MYELOID

ACUTE LEUKEMIA

LYMPHOID

FAB classification

WHO classification

MYELOID

Classification

Acute lymphoblastic leukemia: (account for 75% of childhood leukemias), malignant or blast cells morphology resemble primitive precursors of lymphoid cells

Acute myeloid leukemia: (account for 25% of childhood leukemia), malignant or blast cells morphologically resembles myeloid precursors

Acute Leukemia Types

In most cases acute leukemia develop for no known reason - idiopathic

Ionizing radiation Cytotoxic drugs Oncogenic viruses: (HTLV-1 , HTLV-2) Genetic factors Chromosomal instability – t(12;21), t(9;22) Immune deficiency

Eitiology

FAB CLASSIFICATION:1. L1: Cells are small, uniform blast; nucleoli are small

and indistinct, cytoplasm is scanty2. L2 : cells are large variable in size; one or more large

nucleoli are present ; cytoplasm is abundant3. L3 : cells are large regular in size and oval or round in

shape , one prominent nucleolus is present, cytoplasm is abundant ,deeply basophilic containing vacuoles.

Classification

WHO Classification

Pathophysiology Manifestation

Malignant Cell Infiltration Liver, Spleen, Lymph Node Enlargement. Any Organ Can Be Involved. Symptoms Of That Organ Involved.

Bone Marrow Infiltration Bone Pain, Osteopenia. Even Pathological Fractures Rarely

Decreased Production Of Normal Cells

Anemia Pallor, Fatigue, Decreased Appetite; CCF

Neutropenia Fever; Risk Of Overwhelming Sepsis

Thrombocytopenia Petechiae, Ecchymoses, Mucosal And Other Bleeding

Clinical Manifestation

Phases of management:1. Intensive/ Induction phase (28 days) Vincristine, daunorubicin, L-asparaginase, prednisolone, intrathecal methotrexate.At 15 days and then at 28 days bone marrow aspiration is performed to see remission.

2. Consolidation phase (4 weeks)Cyclophosphamide, cytarabine with intrathecal MTX

3. Interim maintenance ( 4 weeks)Methotrexate, L-asparaginase

Management

4. Re Intensification (6 weeks)Cyclophosphamide, cytarabine and 4 drugs of 1st phase.

5. Maintenance (2 years)6-mercaptopurine, methotrexate and vincristine.

Hematopoietic stem cell transplantation is reserved for patients with Philadelphia chromosome, who fail to achieve remission at end of induction phase or early hematologic relapse.

Potential problems of general and mental health, functional impairment, and activity limitations.

Increased risk of developing neuropsychological abnormalities with cognitive defects.

Highest level of education and employment tend to be lower.

Late complications of irradiation, if given. Second malignancies. Increased risk of developing neuropsychological

abnormalities with cognitive defects. Many children treated for leukemia do well without any

major difficulties.

Complications

Nature of disease: neoplastic condition, multifactorial. Though it is a grave diagnosis but treatment is available. Treatment is prolong but compliance is mandatory for

complete remission. Childhood ALL is one of the most curable cancers, with

intensive chemotherapy regimens achieving a long-term survival rate of around 90%.

Treatment has its own complications but it is mandatory for survival and benefits weigh out the risks.

Even after complete treatment, child needs to regularly see his doctor to address any remaining issues or long term side effects of disease or treatment.

Counselling

Harriet lane 20th ed. PEDIATRIC LEUKEMIAS. CYRINE-ELIANA HAIDER et al.

Childhood Leukemia.John J. Hutter, MD.

Nelson 20th edition. A Success Story Leading Us to Think Big!Jasmine Kamboj, Rahul Kamboj, Tejas Shah, and Pam Khosla

REFERENCES

Thank You!