Sociedad Argentina de Hematología

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Revista Argentina de Hematología


Volumen:    16    # Number : 2

Publication Date :    Mayo - Agosto    Year:    2012


Authors: Attie M., Cocca A., Basack N., Schwalb G., Drelichman G., Aversa L.

Abstract: Hereditary Spherocytosis (HS) is the most common hereditary defects of red cell membrane and the clinical presentation is a hemolytic anemia in which the defect of spectrin or proteins involved in anchoring of spectrin to the membrane, lead to a reduction in red cell surface, resulting in a selective sequestration by the spleen and a shortened red cell life span erythrocyte shorter half-life. Several genes encoding membrane proteins of the cytoskeleton of the red cell are known. Different molecular mechanisms may generate the same defect of membrane protein in HS. The particular protein defect does not influence the clinical management of patients but may confirm the mode of transmission of the disease. The clinical severity varies from asymptomatic to severe hemolysis. The osmotic fragility test, acidified glycerol lysis and autohemolysis have low sensitivity and specificity, so, other studies should be performed in mild or atypical presentation. The treatment is consider with folic acid supplementation. Splenectomy is indicated in severe forms and may be considered in moderate forms.

Key words: Spherocytosis, Hemolysis, Erythrocyte

Pages : 106-113

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