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Purpura, Thrombotic Thrombocytopenic MeSH Descriptor Data 2026
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.
Entry Term(s)
Congenital Thrombotic Thrombocytopenic Purpura
Familial Thrombotic Thrombocytopenia Purpura
Familial Thrombotic Thrombocytopenic Purpura
Microangiopathic Hemolytic Anemia, Congenital
Moschcowitz Disease
Moschkowitz Disease
Purpura, Thrombotic Thrombopenic
Schulman-Upshaw Syndrome
Thrombotic Microangiopathy, Familial
Thrombotic Thrombocytopenic Purpura
Thrombotic Thrombocytopenic Purpura, Congenital
Thrombotic Thrombocytopenic Purpura, Familial
Upshaw Factor, Deficiency of
Upshaw-Schulman Syndrome
Public MeSH Note
1986; see PURPURA, THROMBOTIC THROMBOPENIC 1965-1985
History Note
1986; use PURPURA, THROMBOTIC THROMBOPENIC 1965-1985
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.