Thrombotic Thrombocytopenic Purpura

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

Hemic and Lymphatic Diseases: 6
- Hematologic Diseases: 3366
  - Thrombophilia: 5406
    - Thrombotic Thrombocytopenic Purpura: 3662
  - Cytopenia: 2582
    - Thrombocytopenia: 26776
      - Thrombotic Microangiopathies: 2595
        Thrombocytopenic Purpura: 847
        Thrombotic Thrombocytopenic Purpura: 3662
  - Blood Coagulation Disorders: 167
    - Purpura: 3221
      - Thrombocytopenic Purpura: 847
        Thrombotic Thrombocytopenic Purpura: 3662
  - Blood Platelet Disorders: 126
    - Thrombocytopenia: 26776
      - Thrombotic Microangiopathies: 2595
        Thrombocytopenic Purpura: 847
        Thrombotic Thrombocytopenic Purpura: 3662
Signs and Symptoms Pathological Conditions
- Pathologic Processes: 10023
  - Hemorrhage: 140190
    - Purpura: 3221
      - Thrombocytopenic Purpura: 847
        Thrombotic Thrombocytopenic Purpura: 3662
- Signs and Symptoms
  - Skin Manifestations: 753
    - Purpura: 3221
      - Thrombocytopenic Purpura: 847
        Thrombotic Thrombocytopenic Purpura: 3662

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Disease

Drug/Important Bio-Agent (IBA)