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 Values for Children
Investigations and interpretation
Basic principals of evaluation of anemia's
and disorders of blood
4. 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
5. 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)
17. 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
18. 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
19. 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
20. Increased Destruction of RBC
• Intra corpuscular
– Membrane
– Enzyme
– Hemoglobin
• Extra corpuscular
– Autoimmune
– Fragmentation
– Hyper spleenism
– Plasma factors
21. 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
22. Clinical approach to a child with
anemia
1. History and Examination
2. First Line Investigations
3. Second Line Investigations
23. 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
24. 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
25. 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
27. 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
28. 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
29. 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
30. 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)
32. 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)
33. 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
34. 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)
35. 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
36. 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
37. 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
