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.
Also Known As:
Purpura, Thrombotic Thrombocytopenic; Congenital Thrombotic Thrombocytopenic Purpura; Familial Thrombotic Thrombocytopenia Purpura; Familial Thrombotic Thrombocytopenic Purpura; Microangiopathic Hemolytic Anemia, Congenital; Moschcowitz Disease; Schulman-Upshaw Syndrome; Thrombotic Microangiopathy, Familial; Upshaw Factor, Deficiency of; Upshaw-Schulman Syndrome; Familial Thrombotic Microangiopathy; Microangiopathy, Familial Thrombotic; Schulman Upshaw Syndrome; Thrombocytopenic Purpura, Thrombotic; Thrombopenic Purpura, Thrombotic; Thrombotic Thrombopenic Purpura; Upshaw Schulman Syndrome; Moschkowitz Disease; Purpura, Thrombotic Thrombopenic; Thrombotic Thrombocytopenic Purpura, Congenital; Thrombotic Thrombocytopenic Purpura, Familial