The Hereditary spherocytosis reference article from the English Wikipedia on 24-Apr-2004 (provided by Fixed Reference: snapshots of Wikipedia from wikipedia.org)

Hereditary spherocytosis

Hereditary Spherocytosis is an Hereditary disorder characterized by the presence of spherocytes in the blood smear. Erythrocytes become spherocytes when the connections between the cytoskeleton and the membrane are disrupted. This connections are maintained by an intricate network of proteins, and if one of these proteins is missing normally the discocyte form is altered. Not all proteins deficits lead to spherocytosis, normally ankyrin/spectrin deficits are the most common deficits in spherocytosis, followed by Band 3 deficits and in Japanese populations protein 4.2 deficits are common. Other protein deficits can cause Hereditary Elliptocytosis, Pyropoikilocytosis or Stomatocytosis. Spherocytes can also be present in other conditions not related to protein deficits as for example Autoimmune Haemolytic Anaemia (AIHA).

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