hereditary spherocytosis

hereditary spherocytosis

      congenital blood disorder (blood disease) characterized by an enlarged spleen, spherical (rather than disk-shaped) red blood cells (erythrocyte) of variable size and increased fragility of cell membrane, and a chronic, mild hemolytic anemia punctuated by episodes of severe aplastic anemia (failure of bone marrow to produce cells). Hereditary spherocytosis, the most frequent of the familial anemias, is inherited as an autosomal dominant trait; it is most common among people of northern European descent. Symptoms may be present at birth or may not appear until old age; the disease is most often identified during childhood or adolescence. Removal of the spleen results in cure of anemia in nearly all cases; the defects in blood production remain but do not cause serious disability.

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Universalium. 2010.

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  • hereditary spherocytosis — n a disorder of red blood cells that is inherited as a dominant trait and is characterized by anemia, small thick fragile spherocytes which are extremely susceptible to hemolysis, enlargement of the spleen, reticulocytosis, and mild jaundice * *… …   Medical dictionary

  • Hereditary spherocytosis — This article is about aspects of spherocytosis specific to the hereditary form of the disorder. For details that apply generally to this variant as well as others, see Spherocytosis. Hereditary spherocytosis Classification and external resources… …   Wikipedia

  • Hereditary spherocytosis (HS) — A genetic disorder of the red blood cell membrane clinically characterized by anemia, jaundice (yellowing) and splenomegaly (enlargement of the spleen). In HS the red cells are smaller, rounder, and more fragile than normal. The red cells have a… …   Medical dictionary

  • HS (hereditary spherocytosis) — A genetic disorder of the red blood cell membrane clinically characterized by anemia, jaundice (yellowing) and splenomegaly (enlargement of the spleen). In HS the red cells are smaller, rounder, and more fragile than normal. The red cells have a… …   Medical dictionary

  • Hereditary elliptocytosis — Classification and external resources Blood smear showing elliptocytes ICD 10 D …   Wikipedia

  • Spherocytosis — Infobox Disease Name = PAGENAME Caption = DiseasesDB = 12289 ICD10 = ICD10|D|58|0|d|55 ICD9 = ICD9|282.0 ICDO = OMIM = 182900 OMIM mul = OMIM2|270970 MedlinePlus = 000530 eMedicineSubj = med eMedicineTopic = 2147 MeshID = Spherocytosis is an auto …   Wikipedia

  • Spherocytosis, hereditary (HS) — A genetic disorder of the red blood cell membrane clinically characterized by anemia, jaundice (yellowing) and splenomegaly (enlargement of the spleen). In HS the red cells are smaller, rounder, and more fragile than normal. The red cells have a… …   Medical dictionary

  • spherocytosis — Presence of sphere shaped red blood cells in the blood. SYN: microspherocytosis. [spherocyte + G. osis, condition] hereditary s. [MIM*182900] a congenital defect of spectrin [MIM*182860], the main component of the …   Medical dictionary

  • spherocytosis — n. the presence in the blood of abnormally shaped red cells (spherocytes). Spherocytosis may occur as a hereditary disorder (hereditary spherocytosis) or in certain haemolytic anaemias …   The new mediacal dictionary

  • spherocytosis — A condition in which erythrocytes lose their biconcave shape and become spherical. It occurs as cells age, and is also found in individuals with abnormal cytoskeletal proteins, (hereditary spherocytosis, a disorder that leads to haemolytic… …   Dictionary of molecular biology

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