paediatrichematology-131212124618-phpapp02.pptx

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1. Dr Rasnayaka M Mudiyanse MBBS.DCH.MD.MRCP Consultant pediatrician Teaching Hospital Peradeniya Paediatric Hematology

2. Scope of Pediatric Hematology Introduction (1) Red Cells Anemias of Inadequate Production (2) Hemolytic anemia (3) Thalasaemia (4) Platelets (5) and Coagulation (5) White cells and other topics (6) Pediatric oncology (7)

3. Objectives of the Introductory Lecture Understand the Hematopoietic System Normal Investigation V alues for Children Investigations and interpretation Basic principals of evaluation of anemia's and disorders of blood

4. Phases of Development of the H aemophoitic System Mesoblastic – (Yolk sack) From 10 -14 d of gestation up to 10 -12 wk IU Hepatic From 6 -8 wk of gestation up to 20 -24 wk IU 80% of cells contribute for erythroid series More macrophage than granulocytes ( same as marrow ) Myeloid Extra uterine 40% of the cells contribute for erythroid series

5. Hematologic Values During infancy and Childhood

6. Hematologic Values During infancy and Childhood Age Hb Gr /dl HCT Retic % MCV WBC /mm3 N % L % Cord 16.8 55 5 110 18 000 61 31 2 wk 16.5 50 1 12 000 40 63 3 mo 12 ( 9.5 – 14.5) 36 1 12 000 30 48 ½ – 6 yr 12 (10.5 – 14) 37 1 70-74 10 000 45 48 7-12 yr 13 38 1 76-80 8 000 55 38 Adult female 14 42 1.6 80 7 500 55 (35- 70) 35 Adult male 16 47 80

7. 5 liters of blood in an adult (in a Child 80ml/kg)

8. 5 million red blood cells in 1ml of blood

9. 250 million Hemoglobin molecules in one red blood cell

10. 5 lt of blood in an adults 80ml/kg in a child 5 million red cells in 1 ml of blood 250 million Hemoglobin molecules in one red cell

11. 5 ‘ Chromosome 11 - beta like chains 3’ 5 ‘ Chromosome 16 - alpha like chains 3’

12. Definition of Anaemia Hemoglobin level below the normal range for the age and sex Neonate < 14 gr /dl 1-12 months < 10 gr /dl 1-12 years < 11gr/dl Hemoglobin at birth – cord blood = 16-18 gr /dl 24-48 hour of age – Increase due to hemoconcentration 3 months of age - Drop up to 9.5 – 11 gr /dl

13. Causes of Anemia Impaired Production of RBC Red Cell Aplasia Ineffective erythropoiesis Increased Destruction of RBC Intra corpuscular Extra corpuscular Blood Loss ( overt or apparent) Internal External

14. Impaired Production of RBC Red cell aplasia Parvovirus B 19 infection, Diamond- Blackfan , Transient erythroblastopenia of childhood, Fanconi anaemia, A plastic anaema , L eukemia Ineffective erythropoiesis Iron/ folic acid deficiencies, Chronic Inflammation CRF Myalodisplasia Lead poisoning

15. Increased Destruction of RBC Intra corpuscular Membrane Enzyme Hemoglobin Extra corpuscular Autoimmune Fragmentation Hyper spleenism Plasma factors

16. Causes of Blood Loss Intra uterine Feto -maternal bleeding Feto -fetal bleeding Chronic gastrointestinal blood loss Meckel’s diverticulum, GOR Peptic ulcer disease Hook worm manifestation Inherited bleeding disorders - von Wilebrand’s disease Chronic hematuria

17. 1. History and Examination 2. First Line Investigations 3. Second Line Investigations Clinical approach to a child with anemia

18. Clinical approach to a child with anemia – History and Examination Family History Evidence bleeding ( overt and hook worm, urine) Evidence of hemolysis ( Jaundice, pallor) Involvement of other cell line ( petechea , infections) Dysmorphic features

19. Clinical approach to a child with anemia – Investigations First line investigations FBC Retic count SBR Blood picture Iron/vitamin status Bone Marrow Biopsy HPLC/Hb Electro.. RBC enzymes Immune studies Genetic studies Radio isotopes

20. SBR in anemia with high or normal reticulocyte count SBR normal Blood loss Treated iron deficiency SBR High  Hemolysis Look at the blood picture MCV and MCH related Specific

21. Blood Picture in Anemia Spherocytosis Hereditary , Autoimmune Hemolysis, Wilsons Sickle Cells Sickle or beta sickle Target cells Hemoglobin SC disease Micro angiopathic blood picture HUS, thrombotic thrombocytopenia Bite Cells – G6PD deficiency

22. Blood Picture or FBC Report in patients with normal or high reticulocyte Counts Microcytic ( MCV less than 80) Iron deficiency Thalassemia – α , β ,& E Lead poisoning Sideroblastic anemia Macrocytic ( MCV more than 100) Megaloblastic + Folate /B12 deficiency No megaloblast – Diamond - Blackfan , congenital dyserythropoietic anemia Normocytic ( MCV 80-100) Chronic disease Renal disease Transient erythroblastopenia of child hood ( TEC) Anemia associated with hypothyroidism

23. Beta Thalassaemia major – Only Hb F present Alpha Thalassaemia – HPLC normal Beta Thalassaemia trait – Increased HbA 2 Sickle disease – Hbs – No HbA present

24. Anemia of Inadequate Production Congenital Hypo plastic Anemia ( Diamond - Blackfan ) Pearson Marrow – Pancrease syndrome Acquired Pure Red Blood Cell Anemia Anemia of chronic Disorders and renal disease Congenital Dyserythropoietic Anemias Physiologic anemia of infancy Megaloblastic anemia Iron deficiency anemia

25. 3 year old boy presented with fatigue and shortness of breath for two weeks duration. He is pale and jaundiced. Results of his initial investigations are as follows; Hb – 6.5 gr /dl, Reticulocute count 6%. What is your DD (15) Tabulate other clinical features that you would like to elicit in order to arrive at a diagnosis and their relevance in a table (45) Tabulate investigations and their relevance (40)

26. DD Hemolytic anemia Membrane defects – hereditary Spherocytosis Enzyme defect - G6 PD deficiency Extracellular causes – Immune hemolytic anaemia Thalassaemia – Beta thalasaemia intermedia or E-beta thalasaemia Blood loss Treated for iron deficiency

27. 3 year old boy presented with fatigue and shortness of breath for two weeks duration. He is pale. Results of his initial investigations are as follows; Hb – 5.5 gr /dl, Reticulocute count 0.5 %. What is your DD (15) Tabulate other clinical features that you would like to elicit in order to arrive at a diagnosis and their relevance in a table (45) Tabulate investigations and their relevance (40)

28. DD for Anaema with low retic count Congenital Aplastic anaemia Fanconi Pure red cell aplasia Acquired bone marrow suppression Infections – Parvo virus Drugs – radiation, chemotheraphy Bone marrow infiltration – Leukemia or other infiltrative disease

29. 3 year old boy admitted from the OPD for evaluation. He is pale. Hb – 5.5 gr /dl List 5 features elicited from the history that help to arrive at a diagnosis (30) Tabulate 5 clinical examination finding that help to arrive at a diagnosis and their relevance in a table (30) List 5 initial investigations and their relevance (40)

30. Clinical feature in history Relevance 1. Family history of inherited anaemia Consider Thalassaemia, Hereditary Spherocytosis, 2. Birth weight maturity, iron supplement Anaemia of prematurity . Iron deficiency anaemia 2. Dark urine Intra vascular Hemolysis , hematuria 2. History of bleeding rectal , vomiting, hematuria Anaemia due to blood loss 3. Dietetic history Nutritional anaemia 4. Jaundice Hemolytic anemia 5. Bruises and echymosis Evidence of low platelets 6. Recurrent infections Involvement of white cell line 7. Taking drugs Bone marrow suppression 8. General ill health, bone pain and fever Leukemia

31. Clinical feature in examination Relevance 1. Dismorphic features Fancon i anemia other inherited aplastic anaemia 2. Jaundice Haemolytic anaemia 3. Hepatomegaly Hemolytic anaemia, Leukemia 4. Splenomegaly Hemolytic anaemia, Leukemia Very large spleen - Hyperspleenism 5. Patichie and echymoses Low platelets; Bone marrow suppression, leukemia 6. Bone tenderness Leukemia 7. Emaciated and Chronically ill looking Anaemia of chronic disease or CRF 8. Acute and critically ill child DIC, hemolytic disease

32. Investigation Relevance 1. Hb Establish base line Hb , consider need for transfusion 2. Reticulocyte count Low count – bone marrow failure High count – Hemolysis or treated iron deficiency Normal count – deficiency anaemia 3. Full blood count Involvement of other cell lines - Aplastic anaemia Abnormal cells - leukemia 4. Serum bilirubin Increase – Hemolysis 5. Blood picture Leukemia, Categorize Microcytic, Normocytic and Macrocytic , Red cell morphology 6. HPLC or Hb Electrophoresis To establish diagnosis in Thalassaemia 7. Bone marrow biopsy Aplastic anaemia, leukemia 8. G-6-PD assay 9. Osmotic fragility test