Resident Report 2/22 -TTP

Teaching Points:

  • TTP is a hematologic emergency. It is manifested by the clinical pentad of:
    • Thrombocytopenia
    • Microangiopathic hemolytic anemia (schistocytes on smear)
    • Acute renal insufficiency
    • Fevers
    • Encephalopathy
  • Only need thrombocytopenia and MAHA in order to make the diagnosis
  • Treatment involves initiation of plasmapheresis
  • ADAMTS 13 Testing
    • ADAMTS 13 functions as a protease that cleaves the von Willebrand multimers
    • Deficiency of ADAMTS 13 leads to accumulating amounts of vWF, causing platelet aggregation (leading to thrombocytopenia) and disruption/breaking of RBCs (MAHA)
    • Activity influenced by plasma products and transfusions.
    • Levels of Activity
      • >50% activity argues against TTP.
      • <10% activity suggests TTP
      • 10-50% activity is an ambiguous area
    • Presence of an ADAMTS13 inhibitor
      • Presence suggested acquired TTP
      • Lack of inhibitor suggests hereditary cause of TTP.

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