Canadian Medical Guide > Diseases > Hemic and Lymphatic Diseases > Hematologic Diseases > Anemia > Anemia, Hemolytic > Anemia, Hemolytic, Congenital Terms and Definitions




Anemia, Hemolytic, Congenital

Medical Definition: Hemolytic anemia due to various intrinsic defects of the erythrocyte.
Anemia, Hemolytic, Congenital Categories.
Canadian Medical Guide Definition Anemia, Dyserythropoietic, Congenital - A familial disorder characterized by anemia with multinuclear erythroblasts, karyorrhexis, asynchrony of nuclear and cytoplasmic maturation, and various nuclear abnormalities of bone marrow erythrocyte precursors. Type II is the most common of the 3 types of congenital dyserythropoietic anemia; it is often referred to as HEMPAS, based on the Hereditary Erythroblast Multinuclearity with Positive Acidified Serum test.
Canadian Medical Guide Definition Anemia, Hemolytic, Congenital Nonspherocytic - Any one of a group of congenital hemolytic anemias in which there is no abnormal hemoglobin or spherocytosis and in which there is a defect of glycolysis in the erythrocyte. In some cases, pyruvate kinase deficiency has been demonstrated; in other cases, glucose-6-phosphate dehydrogenase deficiency has been demonstrated.
Canadian Medical Guide Definition Anemia, Sickle Cell - A disease characterized by chronic hemolytic anemia, episodic painful crises, and pathologic involvement of many organs. It is the clinical expression of homozygosity for hemoglobin S.
Canadian Medical Guide Definition Elliptocytosis, Hereditary - An intrinsic defect of erythrocytes inherited as an autosomal dominant trait. The erythrocytes assume an oval or elliptical shape.
Canadian Medical Guide Definition Glucosephosphate Dehydrogenase Deficiency - A disease-producing enzyme deficiency subject to many variants, some of which cause a deficiency of enzyme activity in erythrocytes, leading to hemolytic anemia.
Canadian Medical Guide Definition Hemoglobin C Disease - A disease characterized by compensated hemolysis with a normal hemoglobin level or a mild to moderate anemia. There may be intermittent abdominal discomfort, splenomegaly, and slight jaundice.
Canadian Medical Guide Definition Spherocytosis, Hereditary - A familial congenital hemolytic anemia characterized by numerous abnormally shaped erythrocytes which are generally spheroidal. The erythrocytes have increased osmotic fragility and are abnormally permeable to sodium ions.
Canadian Medical Guide Definition Thalassemia - A group of hereditary hemolytic anemias in which there is decreased synthesis of one or more hemoglobin polypeptide chains. There are several genetic types with clinical pictures ranging from barely detectable hematologic abnormality to severe and fatal anemia.

Anemia, Hemolytic, Congenital Medical Definitions and Terms

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