spherocytic anemia


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Related to spherocytic anemia: spherocytosis, Congenital Spherocytosis

spherocytic anemia

[sfir′əsit′ik]
autosomal-dominant hemolytic anemia characterized by hemolytic anemia caused by the presence of spherical red blood cells. The cells are fragile and tend to hemolyze in the oxygen-poor peripheral circulatory system. Episodic crises of abdominal pain, fever, jaundice, and splenomegaly occur. Because repeated transfusions are often needed to treat the anemia, hemochromatosis may develop. Splenectomy may then be necessary. Compare congenital nonspherocytic hemolytic anemia. See also elliptocytosis.

he·red·i·tar·y spher·o·cy·to·sis

(hĕr-ed'i-tar-ē sfēr'ō-sī-tō'sis)
A congenital defect of spectrin, the main component of the erythrocytecell membrane, which becomes abnormally permeable to sodium, resulting in thickened and almost spheric erythrocytes that are fragile and susceptible to spontaneous hemolysis, with decreased survival in the circulation; results in chronic anemia with reticulocytosis, episodes of mild jaundice due to hemolysis, and acute crises with gallstones, fever, and abdominal pain.
Synonym(s): familial jaundice, spherocytic anemia.

he·red·i·tar·y spher·o·cy·to·sis

(hĕr-ed'i-tar-ē sfēr'ō-sī-tō'sis) [MIM*182900]
Congenital defect of spectrin, the main component of the erythrocyte cell membrane; results in chronic anemia with reticulocytosis, episodes of mild jaundice due to hemolysis, and acute crises with gallstones, fever, and abdominal pain.
Synonym(s): chronic familial icterus, chronic familial jaundice.