Hemolytic Anemias.

Intrinsic (intracorpuscular) abnormalities of red cells

Disorders of membrane cytoskeleton:

Red cell enzyme deficiencies:

pyruvate kinase deficiency
hexokinase deficiency
glucose 6 phosphate dehydrogenase deficiency

Disorders of hemoglobin synthesis:

Deficient globin synthesis: thalassemia syndromes
Structurally abnormal globin synthesis (hemoglobinopathies): sickle cell anemia, unstable hemoglobins

Acquired membrane defect:

paroxysmal nocturnal hemoglobinuria

Extrinsic (extracorpuscular) abnormalities:

Antibody mediated:

Isohemagglutinins: transfusion reactions, erythroblastosis fetalis
Autoantibodies: idiopathic (primary), drug-associated, systemic lupus erythematosus, malignant neoplasms, mycoplasmal infection

Mechanical trauma to red cells:

Microangiopathic hemolytic anemias( MAHA): thrombotic thrombocytopenic purpura, disseminated intravascular coagulation
Prosthetic heart valves

Infections: malaria, hookworm:

Chemical injury: lead poisoning

Sequestration in mononuclear phagocyte system: hypersplenism

Impaired Red Cell Production

Disturbance in stem cells:
aplastic anemia
pure red cell aplasia
anemia of CRF

Disturbance of proliferation and maturation of erythroblasts:

Defective DNA synthesis:
deficiency vitamin B12 and folic acid (megaloblastic anemias)
Defective hemoglobin synthesis
Deficient heme synthesis: iron deficiency
Deficient globin synthesis: thalassemias

Unknown or multiple mechanisms:
sideroblastic anemia
anemia of chronic infections
myelophthisic anemias due to marrow infiltrations


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