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DeCS
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Descriptor English:
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Purpura, Thrombotic Thrombocytopenic
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Descriptor Spanish:
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Púrpura Trombocitopénica Trombótica
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Descriptor Portuguese:
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Púrpura Trombocitopênica Trombótica
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Synonyms English:
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Congenital Thrombotic Thrombocytopenic Purpura
Familial Thrombotic Microangiopathy
Familial Thrombotic Thrombocytopenia Purpura
Familial Thrombotic Thrombocytopenic Purpura
Microangiopathic Hemolytic Anemia, Congenital
Microangiopathy, Familial Thrombotic
Moschcowitz Disease
Moschkowitz Disease
Purpura, Thrombotic Thrombopenic
Schulman Upshaw Syndrome
Schulman-Upshaw Syndrome
Thrombocytopenic Purpura, Thrombotic
Thrombopenic Purpura, Thrombotic
Thrombotic Microangiopathy, Familial
Thrombotic Thrombocytopenic Purpura
Thrombotic Thrombocytopenic Purpura, Congenital
Thrombotic Thrombocytopenic Purpura, Familial
Thrombotic Thrombopenic Purpura
Upshaw Factor, Deficiency of
Upshaw Schulman Syndrome
Upshaw-Schulman Syndrome
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Tree Number:
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C15.378.100.802.687.680
C15.378.140.855.925.750.680
C15.378.925.850
C23.550.414.950.687.680
C23.888.885.687.687.680
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Definition English:
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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. |
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History Note English:
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1986; use PURPURA, THROMBOTIC THROMBOPENIC 1965-1985
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Allowable Qualifiers English:
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Record Number:
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12133
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Unique Identifier:
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D011697
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Occurrence in VHL:
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Similar:
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DeCS
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