An acquired, congenital, or familial disorder caused by PLATELET AGGREGATION with THROMBOSIS in terminal arterioles and capillaries. Clinical features include THROMBOCYTOPENIA; HEMOLYTIC ANEMIA; AZOTEMIA; FEVER; and thrombotic microangiopathy. The classical form also includes neurological symptoms and end-organ damage, such as RENAL FAILURE. Mutations in the ADAMTS13 PROTEIN gene have been identified in familial cases.
Moschcowitz sjukdom — Upshaw-Schulman syndrom — Familjär trombotisk mikroangiopati
Thrombocytopenic Purpura, Thrombotic — Thrombotic Thrombocytopenic Purpura — Moschcowitz Disease — Purpura, Thrombotic Thrombopenic — Thrombopenic Purpura, Thrombotic — Thrombotic Thrombopenic Purpura — Moschkowitz Disease — Familial Thrombotic Thrombocytopenic Purpura — Familial Thrombotic Thrombocytopenia Purpura — Thrombotic Thrombocytopenic Purpura, Familial — Congenital Thrombotic Thrombocytopenic Purpura — Thrombotic Thrombocytopenic Purpura, Congenital — Microangiopathic Hemolytic Anemia, Congenital — Upshaw Factor, Deficiency of — Thrombotic Microangiopathy, Familial — Familial Thrombotic Microangiopathy — Microangiopathy, Familial Thrombotic — Schulman-Upshaw Syndrome — Schulman Upshaw Syndrome — Upshaw-Schulman Syndrome — Upshaw Schulman Syndrome