common haematological problems in children -...
TRANSCRIPT
![Page 1: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/1.jpg)
Paediatric Refresher Course 2017Department of Paediatrics
Sibu Hospital8-9th April 2017
Common Haematological Problems In Children
Dr Quah Shiao Wei
![Page 2: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/2.jpg)
15 month-old, well baby girl, admitted for viral feverFull blood count as follow:Hb : 7.3g/dLMCV : 60 flMCH : 17pgWBC : 10.0 X 10 9/LPlatelet : 460 X 10 9/L
What is the cause of hypochromic microcytic anemia?
![Page 3: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/3.jpg)
Iron Protoporphyrin
Heme
(1) Iron Deficiency Anemia- Nutritional- Blood loss (GIT, menses)
(2) Anemia of inflammation
(3) Sideroblastic Anemia
Globin Haemoglobin
(4) Haemoglobinopathy
(5) Rare : Lead Poisoning
![Page 4: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/4.jpg)
• Onset
- Acute- Chronic
- Incidental finding
• Diet history, Pica
• Medications / Toxic Exposure
• Systemic illness
• Family history
- Aneamia- Regular blood transfusion- Splenectomy
- Gall bladder surgery
• Blood loss (hematochezia, malenic stool)
• Hemolysis (Jaundice, dark-coloured urine)
Focus History
• Growth
• Dysmorphic
• Pallor, Jaundice
• Hepatomegaly, splenomegaly
• Features of skeletal
expansion
- maxillary hyperplasia- frontal bossing
Relevant Physical Examinations
• Peripheral Blood Film / Full
Blood picture
• Iron Studies
• Reticulocyte count
• Hb analysis
Investigations
• Specific Test :
- Stool for occult blood / ova & cyst
- hemolysis workout - retic count- Coomb’s test- bilirubin (total & direct)- LDH- urine for inspection
- haemoglobinopathy- alpha gene DNA analysis
![Page 5: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/5.jpg)
Question 1History
• Fully breastfeeding since
birth till now
• weaning started at 6
month-old
• Diet : mainly
breastfeeding
• Well child
Investigations
PBF: Hypochromic, microcytic, poikilocytosis,
anisocytosis
Serum iron : 1.5 μmol/L (9.0-35.4)
Serum ferritin : 7.5 ug/L (22-322)
Hb F : 1.0% (≤ 1.0%)
Hb A2 : 2.0 (1-3.3%)
Physical Examinations
• weight : 9.3kg, height :70cm
• pallor, not jaundice
• no dysmorphic features
• no organomegaly
Diagnosis ?
Nutritional Iron Deficiency Anaemia
![Page 6: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/6.jpg)
Treatment for Iron Deficiency Anaemia
1) Iron Therapy
T. Ferrous Fumarate 200mg (66mg elemental iron)Treatment dose : 6mg/kg elemental iron per day
Syrup Iron (III) Hydroxide Polymaltose Complex / Maltofer50mg/5ml (1ml= 10mg elemental iron)
Intravenous Iron-Hydroxide Sucrose Complex / Venofer 500mg/5ml- Dosing : 0.5 -2mg/kg (max 100mg)- ≥ 2 year-old- Infusion, diluted in 25ml NS- Give every four weeks, total 12 weeks
2) Diet motification
3) Follow up for the response
- Hb > 1g/dL with four week- Continue at least 3-6 month- deworming
![Page 7: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/7.jpg)
Dietary Sources of Iron
![Page 8: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/8.jpg)
Treatment for Iron Deficiency Anaemia
1) Iron Therapy
T. Ferrous Fumarate 200mg (66mg elemental iron)Treatment dose : 6mg/kg elemental iron per day
Syrup Iron (III) Hydroxide Polymaltose Complex / Maltofer50mg/5ml (1ml= 10mg elemental iron)
Intravenous Iron-Hydroxide Sucrose Complex / Venofer 500mg/5ml- Dosing : 0.5 -2mg/kg (max 100mg)- ≥ 2 year-old- Infusion, diluted in 25ml NS- Give every four weeks, total 12 weeks
2) Diet motification
3) Follow up for the response
- Hb > 1g/dL with four week- Continue at least 3-6 month- deworming
![Page 9: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/9.jpg)
10month-old, well baby boy, referred for pallor.O/E: pale, tinge of jaundice. Liver 2cm, spleen 1cm
Hb : 6.0g/dLMCV : 54 flMCH : 16 pgWBC : 10.0 X 10 9/LPlatelet : 360 X 10 9/L
What is the cause of anemia?
PBF : Hypochromic, microcytic anemia
Serum iron : 12 μmol/L (9.0-35.4)
Serum ferritin : 17 ug/L (22-322)
Hb F : 98.2% (≤ 1.0%)
Hb A2 : 2.3% (1-3.3%)
Beta- Thalassemia Major
![Page 10: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/10.jpg)
Haemoglobin in Human Life
Haemoglobin Type
EmbryonicGower 1 PortlandGower 2
Fetus/ Hb F -22
Adult -Hb A -22
Hb A2-22
![Page 11: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/11.jpg)
Changes in haemoglobin composition in Human Life
![Page 12: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/12.jpg)
Hb analysis Globin Chains Normal Thalassemia major
Hb A 22 > 95%
Hb A2 22 1.5 -3.3%
Hb F 22 <1%
![Page 13: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/13.jpg)
Hb analysis Globin Chains Normal Thalassemia trait
Hb A 22 > 95% 80-90%
Hb A2 22 1.5 -3.3% > 4.0%
Hb F 22 <1% 1-7% (variable)
![Page 14: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/14.jpg)
Microcytic Hypochromic Anemia (MCH < 27pg)
Iron Deficiency Anemia (reduced RBC production)
Thalassemia Trait (ineffective erythropoesis)
Iron Deficiency Anemia Thalassemia Trait
Hb Moderate to severe 9-10g/dL
RDW High Low
Red Cell Distribution Width (RDW)– measure red cell variation
(normal 11-15%)
Mentzer Index (MCV/RBC)
>13 <13
Vs
![Page 15: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/15.jpg)
3 year-old boy, referred for pallor.O/E: pale, tinge of jaundice. mild frontal bossing Liver 2cm, spleen 2cm
Hb : 7.2g/dLMCV : 70 flMCH : 16 pgWBC : 7.0 X 10 9/LPlatelet : 250 X 10 9/L
What is the cause of anemia?
PBF : Hypochromic, microcytic anemia
Serum iron : 12 μmol/L (9.0-35.4)
Serum ferritin : 170 ug/L (22-322)
Hb F : 46.0% (≤ 1.0%)
Hb A2 : 48.2% (1-3.3%)
![Page 16: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/16.jpg)
Hb analysis Globin Chains Normal Hb E- Thala Hb E trait
Hb A 22 > 95%
Hb A2 22 1.5 -3.3% > 25% > 25%
Hb F 22 <1% normal
When Hb A2 > 10%, think about structural variant share the mobility as A2 : Hb E, C, O
Hb E point mutation at 26, glutamic acid to lysine
![Page 17: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/17.jpg)
Hb analysis Patient Father Mother Brother
Hb (g/dL) 7.2 12.0 10.5 11.5
MCV (fl) 70 72 70 72
MCH (pg) 16 22 20 22
Hb A2 48.2% 32.0% 4.5% 35.0%
Hb F 46.0% 2.0% 1.0% 2.0%
Family Screening
Hb E-thala
Hb E trait
Hb trait
Hb E trait
![Page 18: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/18.jpg)
5 year-old girl, referred for pallor.O/E: pale, tinge of jaundice. Liver 1cm, spleen 1cm
Hb : 8.4 g/dLMCV : 67 flMCH : 24 pgSerum Iron & ferritin : normalHb A : 95%Hb A2 : 2.5%Hb F : 0.8%
What is the cause of anemia? Incubated with methylene blue
“ Golf ball”- Hb H inclusion
Hb H disease
Hb Analysis
![Page 19: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/19.jpg)
Normal : ( / )
(1) Alpha plus (+) : (_ / )- silent carrier
Hb adana : CD59
Codon 59 : GGC -> GAC
(2) Alpha zero (0) : (_ _ / )- alpha thal carrier, in Asian( _ / _)- alpha thal carrier, in African
(B) Alpha non-deletional (ND
/ )
Hb constant spring : CS
Codon 142 : TAA -> CAA
(A) Alpha deletion
Alpha Thalassaemia
(3) 3 genes affected (_ _ / _ ) - Hb H Disease
(4) 4 genes affected (_ _/_ _)- Hb Barts
- Severe intrauterine anemia- Hepatopslenomegaly- Generalized edema, ascites- Skeletal & CVS malformation
Hb H-Constant Spring(CS / _ _)
Hb Barts Hydrops Fetalis Phenotype- Inactivations of the alpha globin
e.g. : (ND/ ND)(_ _/ ND)
Inactivation of alpha globin
![Page 20: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/20.jpg)
Molecular diagnosis is important
Alpha thalassemia need DNA analysis!
![Page 21: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/21.jpg)
Thalassaemia Screening For General Population
MCH ≤ 27 pgHb ≤ 9g/dL
Treat For IDA for 3 months
Repeat FBC
MCH> 27 pgnormal
MCH ≤ 27 pg
Proceed with Hb analysisLook at Hb A2 (normal 1.5-3.3%)
Hb A2 ≥ 4%
Thalassaemia Carrier
Hb A2 3.4-3.9%
DNA analysis for β mutation
Β mutation detected
(1) General rules: Hb A2 ≥ 4% marker for trait
![Page 22: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/22.jpg)
Thalassaemia Screening For General Population
MCH ≤ 27 pg
Proceed with Hb analysisLook at Hb A2 (normal 1.5-3.3%)
Hb A2 ≥ 4%
Thalassaemia Carrier
Hb A2 3.4-3.9%
DNA analysis for β mutation
Β mutation detected
Hb A2 ≤ 3.3%MCH <25 pg
αThalassaemiaCarrier
Hb E variant present
Ethnic: non-Indian, i.e. Chinese, Malay, Indigeneous, mixed parentage
DNA analysis for α mutation
α mutation detected
Hb E <25
αThalassaemia& Hb E Carrier
Hb E ≥25= Hb E carrier
![Page 23: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/23.jpg)
Pale & Jaundice
![Page 24: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/24.jpg)
8 year-old girl, well, admit for pallor and jaundice for 3 days, no liver, spleen 3cm
Hb : 3.5 g/dLMCV : 115 flWBC : 11.0 X 10 9/LPlatelet : 170 X 10 9/LAbsolute Retic count: 200Total bilirubin : 60
AnemiaReticulocytosisHyperbilirubinemia
3 cardinal signs of hemolysis
Absolute reticulocyte count (X109/L)= retic count % X RBC X 10
< 20 – poor reticulocyte response20-80 – preserved reticulocyte response> 80 – brisk reticulocyte response
![Page 25: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/25.jpg)
- Direct comb’s test / Direct Antiglobulin Test
To test antibody & complement on RBC surface
Approach to haemolytic anaemia
Immune or non-immune hemolysis ?
![Page 26: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/26.jpg)
8 year-old girl, well, admit for pallor and jaundicefor 3 days, no liver, spleen 3cm
Hb : 3.5 g/dLMCV : 115 flWBC : 11.0 X 10 9/LPlatelet : 170 X 10 9/LAbsolute Retic count: 200Total bilirubin : 60Direct Coomb’s Test: Positive, Ig G
Warm Immune Hemolytic Anemia
What is the cause of anemia?
Ig G Complement C3d Ig G- C3d
Positive Direct Coomb’s Test –Immune Hemolytic Anemia
Cold AIHAParoxysmal Cold Haemoglobinuria
Connective Tissue Disease
Classic Warm AIHA
![Page 27: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/27.jpg)
1 year-old girl, noted pallor during immunizationo/e : pallor, tinge of jaundice, liver 2cm, spleen 2cm
Hb : 6.0 g/dLMCV : 115 flWBC : 10.0 X 10 9/LPlatelet : 170 X 10 9/LAbsolute Retic count: 150Total bilirubin : 50Direct’s Coomb’s Test : negative
What is the cause of hemolytic anemia ?
![Page 28: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/28.jpg)
Non-Immune Hemolytic Anemia
RBC membrane disorder RBC enzyme deficiencies
Ineffective erythropoiesis
Hereditary Spherocytosis
Hereditary Elliptocytosis
Pyruvate KinaseG6PD deficiency
Osmotic fragility test
Haemoglobinopathy
![Page 29: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/29.jpg)
1 year-old girl, noted pallor during immunizationo/e : pallor, tinge of jaundice, liver 2cm, spleen 2cm
Hb : 6.0 g/dLMCV : 115 flWBC : 10.0 X 10 9/LPlatelet : 170 X 10 9/LAbsolute Retic count: 150Total bilirubin : 50Direct’s Coomb’s Test : negative
What is the cause of hemolytic anemia ? Hereditary Spherocytosis
![Page 30: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/30.jpg)
Summary – Approach to Pale Child
1) Is the child in shock (poor perfusion) ? Or anemia ?
2) If anemia , micocytic / normochromic or macrocytic anemia ?
3) Focus history, physical examination & investigations
4) Screening for thalassemia
(1) General rules: Hb A2 ≥ 4% marker for trait
(2) If MCH <25 pg, Hb A2 ≤ 3.3%No marker for thala trait- need to do DNA analysis
5) Cardinal sign of hemolytic anemia : ↓Hb + ↑retic + ↑bilirubin- determine immune or non-immune
![Page 31: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/31.jpg)
Bleeding child !!!
![Page 32: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/32.jpg)
Approach to bleeding child
1) Well Vs Unwell (haemodynamic instability)
2) Timing of bleeding
Immediate bleeding
Delayed bleeding
Defect in primary hemostasis Vascular abnormaly
Defect in secondary hemostasis
![Page 33: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/33.jpg)
Hemostasis
Primary hemostasisFormation of platelet plug
Defect in primary hemostasis- Platelet disorders- Von-Willebrand disease- Immediate bleeding- Mucocutaneous bleeding- Internal bleeding
Secondary hemostasisFormation of cross-linked fibrin to stabilize the platelet plug
Defect in secondary hemostasis- Factors deficiency- Type III vWD- Delayed bleeding- Deep muscle and joint bleeding- Internal bleeding
![Page 34: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/34.jpg)
Significant bleeding
1) Epistaxis not stopped by 10 minutes compression or required medical intervention
2) Cutaneous haemorrhage or bruising without apparent trauma
3) Prolonged bleeding (> 15 minutes) for trivial wounds, or in oral cavity, or recurring spontaneously
within 7 days
4) Post-operative bleeding
5) Menorrhagia leading to anemia
![Page 35: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/35.jpg)
Approach to bleeding child
1) Well Vs Unwell (haemodynamic instability)
2) Timing of bleeding
Immediate bleeding
Delayed bleeding
Defect in primary hemostasis Vascular abnormaly
Defect in secondary hemostasis
3) Site of bleeding
- Mucocutaneous bleeding (oromucosal, nasal, subconjunctival, skin, GI)
- Deep soft tissue bleeding (muscle, joints)
- Internal haemorrhage (intracranial haemorrhage)
4) Severity of bleeding
![Page 36: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/36.jpg)
Site of Bleeding
1) Petechiae (<3mm)
2) Ecchymosis (>3mm)
Mucocutaneous bleeding Deep soft tissue bleeding Internal bleeding
Mucosal
Petechiae
Blister / bullae
Epistaxis
Gingival
Subconjunctival haemorrhage
Skin
Epidermis and dermis
hematoma
Subcutaneous tissue
![Page 37: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/37.jpg)
Site of Bleeding
1) Petechiae (<3mm)
2) Ecchymosis (>3mm)
Mucocutaneous bleeding Deep soft tissue bleeding Internal bleeding
Mucosal Joints
Petechiae
Blister / bullae
Epistaxis
Gingival
Subconjunctival
haemorrhage
hematoma Hemarthosis
Intracranial haemorrhage
Skin
Epidermis and dermis
hematoma
Subcutaneous tissue
Muscle and soft tissue
![Page 38: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/38.jpg)
Approach to bleeding child
1) Well Vs Unwell (haemodynamic instability)
2) Timing of bleeding
Immediate bleeding
Delayed bleeding
Defect in primary hemostasis Vascular abnormaly
Defect in secondary hemostasis
3) Site & severity of bleeding
- Mucocutaneous bleeding (oromucosal, nasal, subconjunctival, skin, GI)
- Deep soft tissue bleeding (muscle, joints)
- Internal haemorrhage (intracranial haemorrhage)
4) Important histories
- Family history- Hemostasis history (spontaneous and provoked)- Drug history
![Page 39: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/39.jpg)
Hemostasis history
Physiological
Neonate- Cord dehiscence
Infancy- Ambulation- Eruption of primary teeth
Neonate- Instrumental delivery- Heel prick bleed- Injection bleeds- vaccination
Childhood and adolescents- Exfoliation of primary teeth- Sport injuries- Menorrhagia
Provoked
Infancy- Vaccinations
Childhood and adolescents- Circumcisions- Dental procedures- Adenoidotonsillectomy- Appendicectomy- Hernia repair
![Page 40: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/40.jpg)
2) Normal platelet, abnormal coagulation profile
1)Isolated thrombocytopenia, normal coagulation profile
4)Normal platelet & normal coagulation profile
a) Prolonged PT b) Prolonged APTTc) Prolonged PT & APTT
Case Senario
3) Thrombocytopenia, abnormal coagulation profile
![Page 41: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/41.jpg)
Slide 41
Isolated thrombocytopenia
![Page 42: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/42.jpg)
3 year-old, well, bruising for 1 week pink, no hepatosplenomegaly or lymphadenopathy
Hb : 13.0 g/dLWBC : 7.0 X 10 9/LPlatelet : 5 X 10 9/LPT : 13APTT : 31TT : 14 (13-16s)Fibrinogen : 2.5 (1.5-4.5g/L)
What is the cause of isolated thrombocytopenia?
![Page 43: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/43.jpg)
Thrombocytopenia
1) True thrombocytopenia ?FBP : Pseudothrombocytopenia due to platelet clumping ?
2) Associated abnormaly ? Look at FBP
• Thrombocytopenia + RBC fragment
→Microangiopathic Hemolytic Anemia
• RBC & WBC precursors? → Bone marrow infiltration
• Circulating blasts? → Acute leukemia
• Atypical lymphoid cells → consider viral infections
![Page 44: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/44.jpg)
Thrombocytopenia
1) True thrombocytopenia ?FBP : Pseudothrombocytopenia due to platelet clumping ?
2) Associated abnormaly ? Look at FBP
• Thrombocytopenia + RBC fragment →Microangiopathic Hemolytic Anemia
• RBC & WBC precursors? → Bone marrow infiltration
• Circulating blasts? → Acute leukemia
• Atypical lymphoid cells → consider viral infections
3) Look at the platelet
• Giant platelet → Bernard- Soulier Syndrome
• Small platelet → Wiskott-Aldrich Syndrome
• WBC inclusion, large platelet → MYH9-related platelet disorders
![Page 45: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/45.jpg)
Thrombocytopenia
1) True thrombocytopenia ?FBP : Pseudothrombocytopenia due to platelet clumping ?
2) Associated abnormaly ? Look at FBP
• Thrombocytopenia + RBC fragment →Microangiopathic Hemolytic Anemia
• RBC & WBC precursors? → Bone marrow infiltration
• Circulating blasts? → Acute leukemia
• Atypical lymphoid cells → consider viral infections
3) Look at the platelet
• Giant platelet → Bernard- Soulier Syndrome
• Small platelet → Wiskott-Aldrich Syndrome
• WBC inclusion, large platelet → MYH9-related platelet disorders
4) If non of above, think of Immune Thrombocytopenia
FBP : large platelet with atypical lymphocytes
![Page 46: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/46.jpg)
3 year-old, well, bruising for 1 week pink, no hepatosplenomegaly or lymphadenopathy
Hb : 13.0 g/dLWBC : 7.0 X 10 9/LPlatelet : 5 X 10 9/LPT : 13APTT : 31TT : 14 (13-16s)Fibrinogen : 2.5 (1.5-4.5g/L)
FBP : large platelet, no abnormal cells
Immune thrombocytopenia
![Page 47: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/47.jpg)
Immune Thrombocytopenia (platelet < 100 X 109/L)
- Immune- mediated thrombocytopenia antiplatelet autoantibody
- Diagnosis of exclusion
- Precipitating factors following viral infections MMR vaccinations (1 in 40,000 vaccinated children)
- Age : 2-10 year-old
![Page 48: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/48.jpg)
Phase of Immune Thrombocytopenia
monthsProvan et al, Blood, 2010; 115:168
![Page 49: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/49.jpg)
Diagnosis of Immune Thrombocytopenia
![Page 50: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/50.jpg)
Investigations in Immune Thrombocytopenia
1) FBC
2) FBP3) Autoimmune screening4) Recommended – Immunoglobulin level (prior to IV IG treatment)
Bone marrow aspiration ?
Consider if
1) Prolonged history of bleeding
2) Additional cytopenia
3) Bone pain
4) Lymphadenopathy
5) Failure to response to treatment
![Page 51: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/51.jpg)
Treatment in Immune Thrombocytopenia
1) Platelet elevating therapy - based on the severity
Treat the patient, not the numbers !
![Page 52: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/52.jpg)
Severity
Non- Severe(cutaneous bleed)
Minor (Grade 1)→ limited petechiae→ < 5 bruises
Mild (Grade 2)→ extensive petechiae→ > 5 bruises
Severe(mucosa bleed)(internal bleed)
Moderate (Grade 3)→ active mucosal bleed→ hematoma
Serious (Grade 4) → internal bleeding
Grade Platelet elevating therapy
I Not recommended, observation only
II Treatment in selected cases
III Recommended
IV Necessary
![Page 53: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/53.jpg)
Treatment
1) Platelet elevating therapy a) Steroidb) IV IGc) Steroid + IV IG
Dose of IV IG
Single dose 0.8g/kg Lancet 1994
1g/kg Blood 1993
Multiple doses 0.8g/kg Day 1, then 1g/kg 72h later Hum immunol 2005
1g/kg for 2 days Blood 1986
0.4/kg for 5 days Lancet 1981
![Page 54: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/54.jpg)
![Page 55: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/55.jpg)
Treatment in Immune Thrombocytopenia
1) Platelet elevating therapy a) Steroidb) IV IGc) Steroid + IV IG
2) Remission inducing therapya) splenectomyb) rituximab
3) Supportive therapya) tranexamic acidb) hormone therapy in menorrhagia
![Page 56: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/56.jpg)
2) Normal platelet, abnormal coagulation profile
1)Isolated thrombocytopenia, normal coagulation profile
4)Normal platelet & normal coagulation profile
a) Prolonged PT b) Prolonged APTTc) Prolonged PT & APTT
Bleeding Child
3) Thrombocytopenia, abnormal coagulation profile
![Page 57: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/57.jpg)
2 year-old boy, spontaneous right knee swelling.
Hb : 9.2 g/dLWBC : 8.0 X 10 9/LPlatelet : 300 X 10 9/LPT : 12APTT : 90TT : 14 (13-16s)Fibrinogen : 2.5 (1.5-4.5g/L)
Prolong APTT, next step ?
After performing mixing test, APTT : 35
Diagnosis ? Factor deficiency
F8 : 1% (80-100%)F9 : 110% (70-120%)VWF Ag: 80% (70-1105)riCoF: vWF: 100% (80-120%)
Mixing test
If corrected, factor deficiency
If not corrected, presence of inhibitor
Isolated prolong APTT – intrinsic pathway
Further ix1st line : FIII, FIX, vWF2nd line : FXI
Diagnosis : Haemophilia A
![Page 58: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/58.jpg)
D9 of life baby, bleeding from umbilical stump for 2 days
Hb : 11.0 g/dLPlatelet : 300 X 10 9/LPT : 100APTT : 34TT : 14 (13-16s)Fibrinogen : 2.5 (1.5-4.5g/L)
Isolated prolong PT
• Vitamin K deficient bleed• Warfarin toxicity
• Rare : FVII deficiency
![Page 59: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/59.jpg)
5 weeks-old Indonesia girl, presented with status epilepticusBirth History : Term baby, born at home. Mother main caretakerO/E: Pupils unequal, all the tones and reflex increased, AF bulging
Hb : 7.0 g/dLWBC : 15.0 X 10 9/LPlatelet : 516 X 10 9/LPT : > 200APTT : > 200TT : 16 (13-16s)Fibrinogen : 1.8 (1.5-4.5g/L)
Diagnosis ?
CT brain
vit k deficient bleeding
![Page 60: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/60.jpg)
2) Normal platelet, abnormal coagulation profile
1)Isolated thrombocytopenia, normal coagulation profile
4)Normal platelet & normal coagulation profile
a) Prolonged PT b) Prolonged APTTc) Prolonged PT & APTT
Bleeding Child
3) Thrombocytopenia, abnormal coagulation profile
![Page 61: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/61.jpg)
5 year-old girl, in ICU, ventilated for severe pneumonia with septic shock
Hb : 8.0 g/dLWBC : 3.0 X 10 9/LPlatelet : 35 X 10 9/LPT : 20 (12-14)APTT : 50 (28-35)TT : 23 (13-16s)Fibrinogen : 0.8 (1.5-4.5g/L)
Diagnosis ?
Disseminated Intravascular Coagulopathy
![Page 62: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/62.jpg)
2) Normal platelet, abnormal coagulation profile
1)Isolated thrombocytopenia, normal coagulation profile
4)Normal platelet & normal coagulation profile
a) Prolonged PT b) Prolonged APTTc) Prolonged PT & APTT
Bleeding Child
3) Thrombocytopenia, abnormal coagulation profile
![Page 63: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/63.jpg)
15 year-old girl, having heavy menses. Presented with giddiness
Hb : 7.5 g/dLPlatelet : 350 X 10 9/LPT : 13 (12-14)APTT : 34 (28-35)TT : 14 (13-16s)Fibrinogen : 1.8 (1.5-4.5g/L)
Next Step ?
Von-willebrand disease
F8 : 100% (80-100%)F9 : 110% (70-120%)VWF Ag: 38% (70-1105)riCoF: vWF: 41% (80-120%)Diagnosis ?
Screen for1) von-Willebrand disease2) Platelet function defect3) FXIII deficiency4) Rare : vitamin c deficiency
![Page 64: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/64.jpg)
Summary to approach to bleeding child
1) Well Vs Unwell
2) Timing of bleeding- immediate Vs delayed
3) Site & severity of bleeding
4) Important histories- Family history- Hemostasis history (spontaneous and provoked)- Drug history
5) Platelet and coagulation profile
![Page 65: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/65.jpg)
Pancytopenia
![Page 66: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/66.jpg)
8 year-old girl, previously well, presented with 1 month history of recurrent bruises, gum bleeding, nose bleeding. She was referred by clinic with the following full blood count:-Hb : 4.6 g/dLMCV : 65 flMCH : 22pgWBC : 3.0 X 10 9/LPlatelet: 3 X 10 9/L
She was pale, not jaundice. There were multiple petechiaes over face, both upper and lower limbs. No organomegaly or lymphadenopathy. She was not dysmorphic, no skeletal abnormalities or pigmentation.
![Page 67: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/67.jpg)
Pancytopenia is an emergency
Bone marrow Infiltration
Acute leukaemia
Bone marrow Failure
Aplastic Anaemia
Immune dysregulation
Haemophagocystic
Lymphohistiocytosis
(HLH)
![Page 68: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/68.jpg)
Thrombocytosis
![Page 69: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/69.jpg)
12 months; well baby; admitted for acute bronchiolitisIncidentally finding of pallor; no hepatosplenomegaly; no lymphadenopathy
Hb (g/dL) 7.1
MCV (fL) 56
MCH (pg) 16.0
WBC (× 109/L) 10.3
Platelet (× 109/L) 1014
What is the cause of thrombocytosis ?
![Page 70: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/70.jpg)
On examinations,
• Bilateral injected conjuctiva, watery non-purulent
• Maculopapular rash on trunk, face, limbs. Blanching. Not itchy.
• No periorbital/perioral swelling
• 1 cervical lymph nodes 1cm X 2cm on right anterior neck
• BCG scar not flared
• No perianal redness or excoriation
• Lips slightly swollen
6 months; previously wellFever 1 week , Cough 4/7
Rash 2/7 appeared on trunk, limbs, faceRed eyes 2/7, Reduced oral intake 1/7
Hb 9.8TWBC 32.3 Plt 917
What is the cause of thrombocytosis ?
![Page 71: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/71.jpg)
ThrombocytosisDefinition: Platelet count > 400 × 109/L
Reactive
• Iron deficiency anemia
• Infection
• Inflammation:• Kawasaki disease• Autoimmune disease
Reactive thrombocytosis does not result in thromboembolic or haemorrhagic complications
Does not required anti-platelet therapy except:1. Kawasaki disease2. Additional thrombotic
risk factors exist
![Page 72: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/72.jpg)
ThrombocytosisDefinition: Platelet count > 400 × 109/L
Reactive Myeloproliferative or Myelodysplastic
Disease
Asplenia
• Iron deficiency anemia
• Infection
• Inflammation:• Kawasaki disease• Autoimmune disease
• Essential thrombocythemia• polycythemia vera (PV)• primary myelofibrosis (PMF)• chronic myeloid leukemia (CML)• 5q- syndrome• RARS with thrombocytosis
Post-splenectomy
![Page 73: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/73.jpg)
12 months; well baby; admitted for acute bronchiolitisIncidentally finding of pallor; no hepatosplenomegaly; no lymphadenopathy
Hb (g/dL) 7.1
MCV (fL) 56
MCH (pg) 16.0
WBC (× 109/L) 10.3
Platelet (× 109/L) 1014
What is the cause of thrombocytosis ?
Thrombocytosis 2°to iron deficiency anaemia
PBF microcytic hypochromicanemia
Iron 1.0µmol/L 9.0-30.4
Ferritin 6.7ug/L 22.0-322.0
HbA2 2.0%
HbF 1.0%
![Page 74: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/74.jpg)
On examinations,
• Bilateral injected conjuctiva, watery non-purulent
• Maculopapular rash on trunk, face, limbs. Blanching. Not itchy.
• No periorbital/perioral swelling
• 1 cervical lymph nodes 1cm X 2cm on right anterior neck
• BCG scar not flared
• No perianal redness or excoriation
• Lips slightly swollen
6 months; previously wellFever 1 week , Cough 4/7Rash 2/7 appeared on trunk, limbs, faceRed eyes 2/7, Reduced oral intake 1/7
Hb 9.8TWBC 32.3 Plt 917
What is the cause of thrombocytosis ?
Kawasaki Disease• Ultrasound 20/12/12 No sonographic evidence
of hydrops of gallbladder• ECHO = Giant aneurysm noted over coronary
arteries
![Page 75: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/75.jpg)
Establishing the cause requires clinical featureshaematological parametersbone marrow aspirate, trephine biopsy, morphological featuresPresence or absence of clonal genetic abnormalities
![Page 76: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/76.jpg)
Reactive Myeloproliferative disease
Asplenia
Famillial / idiopathic Thrombocytosis
Negative Negative Negative
Summary - thrombocytosis
![Page 77: Common Haematological Problems In Children - …hsibu.moh.gov.my/hsb.bm/wp-content/uploads/2017/04/Common... · Common Haematological Problems In Children Dr Quah Shiao Wei. ... Connective](https://reader031.vdocument.in/reader031/viewer/2022022515/5afd0c1b7f8b9a68498c5697/html5/thumbnails/77.jpg)
Thank you