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Updated: Mar 26 2020

Thrombotic Thrombocytopenic Purpura (TTP)

  • Snapshot
    • A 21-year-old woman with past medical history of SLE presents with fever, fatigue, and altered mental status. On physical exam, a petechial rash is seen on her upper extremities. Her temperature is 103°F (39.4°C). Her labs are notable for Hgb 7.3 g/dL, platelets 17,000/mm3, and creatinine of 3.4 mg/dL.
  • Introduction
    • Thrombotic microangiopathy characterized by a pentad
      • microangiopathic hemolytic anemia
      • acute renal failure
      • thrombocytopenia
      • fever
      • neurologic abnormalities
    • Similar to HUS but with fever and neurologic symptoms
    • Epidemiology
      • female > male
      • most commonly in young adults
    • Pathogenesis
      • deficiency or inhibition of metalloproteinase ADAMTS13
        • most commonly disabled by an autoantibody
        • ADAMTS13 degrades vWF multimers
        • deficiency → large vWF multimers → increased platelet adhesion → platelet thrombosis
        • important underlying risk factor, but requires a secondary trigger
      • triggers
        • drugs (ticlopidine, clopidogrel, and cyclosporine)
        • SLE
  • Presentation
    • Symptoms (recall pentad)
      • thrombocytopenia
        • easy bleeding
        • epistaxis
      • neurological symptoms
        • confusion
        • seizures
      • thrombosis
      • renal dysfunction (less so than HUS)
    • Physical exam
      • fever
      • pallor (from anemia)
      • purpura/petechiae
      • jaundice (from hemolysis)
      • splenomegaly
  • Evaluation
    • Complete blood count
      • anemia
      • thrombocytopenia
    • Peripheral blood smear
      • schistocytes (helmet cells)
    • Serum
      • ↑ LDH
      • ↑ creatinine
      • negative Coomb’s
    • ↑ bleeding time
    • normal PT/PTT
  • Differential Diagnosis
    • DIC
    • HUS
    • Sepsis
  • Treatment
    • Plasma exchange transfusion with fresh frozen plasma
    • Steroids
    • Splenectomy
    • Platelet transfusion contraindicated
      • platelet transfusion will worsen disease by feeding the platelet consumption
  • Prognosis, Prevention, and Complications
    • Prognosis
      • remission with plasma exchange in majority of patients
      • mortality rate 13-15%
    • Complications
      • permanent neurologic sequelae
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