An inherited disorder in which there are a large number of unusually small, round red blood cells (spherocytes) in the circulation. These abnormal red cells are readily broken up when blood passes through the spleen. At times, the rate of red cell destruction exceeds the rate at which new cells can be made in the bone marrow, leading to symptoms of anaemia. Other symptoms include jaundice and enlargement of the spleen. Occasionally, crises occur (usually triggered by infection) in which all symptoms worsen. Gallstones are a frequent complication. Splenectomy usually leads to permanent improvement.
Spherocytosis, hereditary |
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