Thrombotic thrombocytopenia purpura

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Background

Pathophysiology

  • Caused by acquired or inherited deficiency of the ADAMTS13 metalloprotease
    • Acquired is via autoantibody
  • ADAMTS13 normally breaks larger von Willebrand factor multimers into smaller particles
  • This deficiency results in increased high-molecular-weight vWF, which aggregates and activates platelets

Clinical Manifestations