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1. Haemolytic anaemias
Atifa Ambreen

2. Introduction
• Mean life span of a RBC-120days
• Removed Extravascularly by- Macrophages of RE system.

3. Intravascular haemolysis occurs in some pathological
disorders

4. Normal red blood cell
breakdown.This takes
place extravascularly
in the macrophagesof
the
reticuloendothelilial
system

5. Hemolytic Anemia
• Definition:
– Those anemias which result from an increase in RBC destruction
• Classification:
– Congenital / Hereditary
– Acquired

6. Classification of Hemolytic Anemias
• Hereditary
Memeberane
Hereditary spherocytosis,Hereditary elliptocytosis
Metabolism
G6PD Deficiency,pyruvate kinase deficiency
Haemoglobin
Genetic abnormalities(Hb S,Hb C,unstable)

7. Acquired
• Immune
Autoimmune
warm antibody type
cold antibody type
Alloimmune
haemolytic transfusion reaction
haemolytic disease of newborn
Allografts,especially stem cell transplantation
Drug associated

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• Red cell fragmentation syndromes
March haemoglobinuria
infections
Malaria,Clostridia
Chemical and physical agents
Especially drugs,burns
Secondary
liver and renal disease
proxysmal nocturnal haemoglobinuria(PNH)

9. Laboratory evaluation of Haemolysis
• HEMATOLOGIC Extravascular Intravascular
Routine blood film Polychromatophilia Polychromatophilia
• Reticulocyte count Increase increase
• Bone marrow examination Erythroid Erythroid
hyperplasia hyperplasia
• PLASMA OR SERUM
• Bilirubin unconjugated unconjugated
• Haptoglobin Dec,Absent Absent
• Plasma hemoglobin N/inc. Increase
• Lactate dehydrogenase Variable increase

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• Extravascular Intravascular
• URINE
• Bilirubin 0 0
• Hemosiderin 0 +
• Hemoglobin 0 + in severe
cases

11. Red Cell Membrane Defects
1.Hereditary Spherocytosis
– Usually inherited as Autosomal Dominent disorder
– Defect: Deficiency of Beta Spectrin or Ankyrin  Loss of membrane
in Spleen & RES becomes more spherical Destruction in Spleen

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13. Clinical features
• Asymptomatic
• Fluctuating hemolysis
• Splenomegaly
• Pigmented gall stones- 50%

14. Clinical course may be complicated with Crisis:
– Hemolytic Crisis: associated with infection
– :Aplastic crisis associated with Parvovirus infection

15. Investigations
– Test will confirm Hemolysis
– Periphral Smear: Spherocytes
– Osmotic Fragility: Increased
Screen Family members

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18. • 2.Hereditary Elliptocytosis
Functional abnormality in one or more anchor proteins in RBC
membrane- Alpha or beta spectrin , Protein 4.1
• Usually asymptomatic

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20. South-East Asian ovalocytosis:
• Caused by a nine amino acid deletion at he junction of the cytoplasmic
and transmemberane domains of the band 3 protein
• Asymptomatic-usually
• Cells oval , rigid ,resist invasion by malarial parasites
• Common in malaysia , indonesia

21. Membrane abnormalities - Enzymopathies
• Deficiencies in Hexose Monophosphate Shunt
– Glucose 6-Phosphate Dehydrogenase Deficiency
• Deficiencies in the EM Pathway
– Pyruvate Kinase Deficiency

22. Glucose 6-Phosphate Dehydrogenase
• Regenerates NADPH, allowing regeneration of
glutathione
• Protects against oxidative stress
• Lack of G6PD leads to hemolysis during oxidative stress
– Infection
– Medications
– Fava beans
• Oxidative stress leads to Heinz body formation, 
extravascular hemolysis

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24. Investigations
–P. Smear: Bite cells, blister cells, irregular small
cells, Heinz bodies, polychromasia
–G-6-PD level

25. 2. Pyruvate Kinase Deficiency
–Deficient ATP production, Chronic hemolytic
anemia
–Investigations
• Priphral Smear: Prickle cells
• Decreased enzyme activity

26. Autoimmune Hemolytic Anemia
• Result from RBC destruction due to RBC autoantibodies: Ig G, M,
E, A
• Most commonly-idiopathic
• Classification
– Warm Autoimmune haemolysis:Ab binds at 37degree Celsius
– Cold Autoimmune haemolysis: Ab binds at 4 degree Celsius

27. 1.Warm Autoimmune hymolysis
•
Usually IgG
Idiopathic
Secondary causes
SLE,CLL,lymphomas,Drugs(e.g.Methyldopa)

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MACROCYTE
SPHEROCYTE
IMMUNOHEMOLYTIC
ANEMIA

29. Investigation
– e/o hemolysis, MCV
– P Smear: Microspherocytosis, n-RBC
– Confirmation: Coomb’s Test / Antiglobulin test

30. Direct Antiglobulin test
demonstrating the presence of autoantibodies
or complement on the surface of the red blood
cell.

31. Cold Autoimmune haemolysis
• Usually IgM
Infections
Mycoplasm pneumonia,infectiousmononucleosis lymphoma
proxysmal cold haemoglobinuria

32. Investigation
• Periphral Smear:
Microspherocytosis
Ig M with specificity to I or I Ag

33. Alloimmune
• Induced by red cell antigens
Haemolytic transfusion reactions
haemolytic disease of the new born
post stem cell grafts
Drug induced
Drug-red cell membrane complex
immune complex

34. THANK YEW ... !!!