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Sickle cell anaemia: peripheral blood film showing deeply staining sickle cells, target cells and polychromasia.
Sickle cell anemia
Sickle cell anemia●Hemoglobin electrophoresis:In Hb SS: No Hb A is detected.The amount of Hb F is variable and is usually 5-15%.
Pathogenesis:Hb S (Hb α2β2S) is insoluble and forms crystals when exposed to low oxygen tension.Deoxygenated sickle hemoglobin polymerizes into long fibres, each consisting of seven intertwined double strands with cross-linking.The red cells sickle and may block different areas of the microcirculation or large vessels causing infarcts of various organs.The sickle β-globin abnormality is caused by substitution of valine for glutamic acid in position 6 in the β chain
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Pathophysiology and morphologic consequences of sickle cell anemia
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