This document discusses hemolytic anemia, which is a type of anemia caused by the premature destruction of red blood cells. It can be intrinsic, due to defects in red blood cell membranes or hemoglobin, or extrinsic, caused by antibodies or the complement system. Some key intrinsic causes mentioned are sickle cell anemia, spherocytosis, and glucose-6-phosphate dehydrogenase deficiency. The mechanism of hemolysis can be extravascular, where red blood cells are destroyed in the spleen or liver, or intravascular, where they are destroyed in the bloodstream. Labs that can indicate hemolytic anemia include decreased haptoglobin, increased lactate dehydrogenase and unconjugated bilirubin levels, and the presence

1. HEMOLYTIC
ANEMIA
DR SEHRISH AHMED

2. Anemia
Acute BloodLoss
Under production
Over destruction
Hemolytic Anemia
Intrinsic
Hb Defect
Sickle Cell
Membrane Defect
Spherocytosis
Enzyme Deficiency
G6PD
Extrinsic
Valve
Complement
Antibodies
CAUSES

4. Erythroid Hyperplasia
Reticulocytosis

5. Decreased Haptoglobin

6. MECHANISM OF
HEMOLYSIS
• Extravascular
• Intra vascular

7. EXTRAVASCULAR
HEMOLYSIS
Destruction of Red Cell by Reticuloendothelial
cells in the liver, spleen and lymph node

8. UCB
LDH

9. Red Cell
Membrane
Defect

10. Hereditary SpherocytosisHowell-Jolly bodies

11. Disorder of
Hemoglobin
Synthesis

12. SICKLE CELL ANEMIA
• Autosomal recessive mutation in B
chain of Hb
• >90% HbS
• Increase risk of sickling
• Hypoxemia
• Dehydration
• Acidosis

14. Hydroxyurea increases level of HbF

15. Thalassemia
Anemia due to decrease synthesis of the globin chains of
hemoglobin
Decrease Globin Decrease Hb Microcytic
Anemia

18. Red Cell Enzyme Deficiency

19. Glucose-6-Phosphate Dehydrogenase
Deficiency
G6PD deficiency is associated with transient episodes of
intravascular hemolysis caused by exposure to an environmental
factor (usually infectious agents or drugs) that
produces oxidant stress.

20. G6PDH NADPH
Reduced
Glutathione
Oxidative Injury
by H2O2
INTRAVASCULAR
HEMOLYSIS

21. Heinz Bodies

22. UCB
LDH
Haptoglobin

23. INTRAVASCULAR
HEMOLYSIS

25. HEMOGLOBINURIA
HEMOSIDERINURIA
HEMOGLOBINEMIA

26. How to read labs?
• Hemolysis “Both”
• Especially intravascularIf haptoglobin is Low
• Both
• Especially ExtravascularIncreased UCB
• BothIncreased LDH
• IntravascularUrine Hb & Hemosiderin
• ExtravascularSplenomegaly

27. Hereditary
Causes of
hemolytic
anemia:
• Abnormality in the cell membrane cytoskeleton
leads to spherocytosis and elliptocytosis.
RBC cell membrane defect:
• G-6-PD deficiency.
• Pyruvate kinase deficiency.
• Hexokinase deficiency
• Glutathione synthetase deficiency
Red cell enzyme deficiency:
• Hemoglobin synthesis defect like Thalassemia.
• Abnormal structure of the hemoglobin-like
Sickle cell anemia, Hb E
Disorders of hemoglobin synthesis:

28. Acquired Causes of hemolytic anemia:
Antibody-mediated
cytotoxic
reaction (Type II) like
ABO incompatibility.
Microangiopathic
hemolytic anemia
Infections: Hemolytic
anemia
Paroxysmal nocturnal
hemoglobinuria.