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  • 1. 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)
  • 2. Objectives of the Introductory Lecture • • • • Understand the Hematopoietic System Normal Investigation Values for Children Investigations and interpretation Basic principals of evaluation of anemia's and disorders of blood
  • 3. Phases of Development of the Haemophoitic 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
  • 4. The Hematopoietic System Pluripotent Stem Cells Capable of self renewal and of clonal maturation in to all blood cell lineage Granulocytopoiesis Thrombopoiesis Megakariocyte progenitors and megakaryocytes G-CSF, M-CSF, GM-CSF, SCF Thrombopoitin TPO Erythropoiesis Erythropoitin (EPO)
  • 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 36 1 12 000 30 48 37 1 70-74 10 000 45 48 76-80 8 000 55 38 80 7 500 55 (35- 70) 35 ( 9.5 – 14.5) ½ – 6 yr 12 (10.5 – 14) 7-12 yr 13 38 1 Adult female 14 42 1.6 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. Organization of Globin Genes 5‘ Chromosome 11 - beta like chains ψβ 5‘ ε GγAγ δβ Chromosome 16 - alpha like chains ζ ψα α1α2 • Gower 1 • Gower 2 • Gower 3 ζ2 ε2 α2 ε2 ζ2 γ2 Fetal • Hemoglobin F α2 γ2 Adult • Hemoglobin A1 • Hemoglobin A2 α2 β2 α2δ2 Embryonic 3’ 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, – Aplastic anaema, – Leukemia • 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. Clinical approach to a child with anemia 1. History and Examination 2. First Line Investigations 3. Second Line Investigations
  • 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. HPLC – High Performance Liquid Chromatography ( Hb electrophoresis can be used ) 1. Beta Thalassaemia major – Only Hb F present 2. Alpha Thalassaemia – HPLC normal 3. Beta Thalassaemia trait – Increased HbA2 4. 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%. 1. What is your DD (15) 2. Tabulate other clinical features that you would like to elicit in order to arrive at a diagnosis and their relevance in a table (45) 3. 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 %. 1. What is your DD (15) 2. Tabulate other clinical features that you would like to elicit in order to arrive at a diagnosis and their relevance in a table (45) 3. 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 1. List 5 features elicited from the history that help to arrive at a diagnosis (30) 2. Tabulate 5 clinical examination finding that help to arrive at a diagnosis and their relevance in a table (30) 3. 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 Fanconi 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