hemophilia (bleeder's disease) (hē´mōfil´ē
),
),n a sex-linked genetic disease manifested in males and characterized by severe hemorrhage.
hemophilia A (classic hemophilia),
n a hemorrhagic diathesis resulting from a deficiency of antihemophilic globulin (AHG); inherited as a recessive sex-linked characteristic and characterized by recurrent bouts of bleeding from even trivial injury. The coagulation time is prolonged, but the bleeding time is normal.
hemophilia B (Christmas disease, hemophilia II, hemophilioid state C),
n a hemorrhagic diathesis resulting from a deficiency of plasma thromboplastin component (PTC); transmitted as a sex-linked recessive characteristic and characterized clinically by the same manifestations as classic hemophilia. There is a delay in the generation of thromboplastin. The platelet count, bleeding time, tourniquet test, and thrombin and prothrombin times are normal.
hemophilia C (plasma thromboplastin antecedent [PTA] deficiency, Rosenthal's syndrome),
n a hemophilia-like condition believed to result from a deficiency of plasma thromboplastin antecedent (PTA), transmitted as a simple autosomal dominant trait and characterized by a moderate bleeding tendency after extraction of teeth or after tonsillectomy. Prothrombin consumption and thromboplastin generation are abnormal. See also factor XI.
hemophilia, classic,
n See hemophilia A.
hemophilia, vascular,
n a hereditary hemorrhagic disorder affecting both sexes and associated with a deficiency of antihemophilic globulin and vascular abnormalities characteristic of pseudohemophilia (von Willebrand disease). The bleeding time is prolonged, and severity of bleeding varies considerably among persons with this condition.
classic hemophilia
see hemophilia A.
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