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Keywords:

  • thrombotic thrombocytopenic purpura;
  • ADAMTS13;
  • exacerbation;
  • relapse

Abstract

Objective:  Approximately 40% of idiopathic thrombotic thrombocytopenic purpura (TTP) patients will suffer an exacerbation (recurrence of TTP within 30 d after their last plasma exchange (PE) procedure), but there are no data to predict who is at greater risk. We studied the clinical utility of demographic and ADAMTS13 biomarker data to predict the risk for exacerbation.

Patients:  Forty-four acute episodes of idiopathic TTP from 26 patients were studied.

Methods:  PE was performed plus either prednisone (1 mg/kg/d) or cyclosporin (2–3 mg/kg/d) as adjuncts. PE was continued daily until response (platelet count >150 000/μL and normalized lactate dehydrogenase) and tapered uniformly in all patients. ADAMTS13 biomarkers were studied prior to PE and after achieving a response, but within 7 d of the last PE.

Results:  African American race (AA) was associated with an increased risk for exacerbation (P = 0.046). ADAMTS13 at presentation was also significantly lower in patients experiencing an exacerbation (P = 0.0364). After adjusting for the race effect, ADAMTS13 remained marginally significant (P = 0.0569).

Conclusions:  AA is significantly associated with an increased risk for exacerbations of TTP. These data also suggest that decreasing pretreatment ADAMTS13 activity was associated with an increased risk for exacerbation, even after accounting for the effect of race.