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Hereditary spherocytosis (HS) is an autosomal, dominantly inherited hemolytic disorder which
shows characteristic spherocytes on peripheral smear. As spherocytes are rounder, more fragile and
susceptible to extravascular hemolysis in the spleen, HS patients suffer from variable degrees of
anemia, jaundice, splenomegaly and gall stones. However the pathogenesis is heterogenous in HS,
such as spectrin deficiency, decreased spectrin-protein 4.1 binding, increased spectrin binding to the
RBC... |
