Thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura TTP

Thrombotic thrombocytopenic purpura

  • Facts:
    Platelet microthrombi block off small blood vessels

    Leads to end organ ischemia and dysfunction

    RBCs get fragmented by contact with microthrombi, leading to hemolysis
  • History / PE:
    Classic HUS triad (thrombocytopenia, ARF, hemolytic anemia ) + fever and neurologic changes (delerium, stroke, seizure)
  • Diagnosis:
    Largely clinical

    Schistocytes on blood smears

    Nucleated RBCs

    Normal coagulation factors

    Elevated indirect bilirubin, LDH, and AST

    Low haptoglobin
  • Treatment:

    Emergent plasmapheresis

    Splenectomy (rare)

    *Platelet transfusions (contraindicated)

    **TTP is fatal in 80% of cases

  • Pneumonic:
    FAT RN

    F ever

    A nemia (hemolytic)

    T hrombocytopenia

    R enal failure

    N eurological abnormalities

  • Notes:
    Type of microangiopathic hemolytic anemia (MAHA), along with HUS, DIC

    Though to be due to either a deficiency or autoantibody against a specific von Willebrand factor-cleaving protease (ADAMTS-13), which causes accumulation of large von WIllebrand factor multimers and platelet aggregation

  • See Also:
    Clotting disorders
  • Differential Diagnosis:
    HUS (classic triad)

    DIC (prolonged PT and aPTT time)

    HELLP (affects pregnant women)

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