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Treatment

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Treatment of acute immune TTP is with both:

  • Urgent plasma exchange (PEX) (1,2)
    • prophylactic plasma infusion remains the mainstay for treatment of hereditary TTP resulting from mutations in ADAMTS13 (2)
    • therapeutic plasma exchange (TPE) is the treatment of choice for acquired TTP due to autoantibodies against ADAMTS13 (2)
  • Immunosuppression – to switch off the immune system response destroying the ADAMTS13 in the Immunosuppression is with high dose steroids initially and Rituximab.

Early administration of rituximab during acute episodes reduces time to remission and rituximab should be started within 72 hours of diagnosis. On average, inpatient stay is 14 days and treatment continues as an outpatient, aiming to normalise ADAMTS13 activity (1)

Reference:

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