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Abstracts from the American society for apheresis 28th annual meeting, april 18–21, 2007 Nashville, Tennessee


Publisher
John Wiley and Sons
Year
2007
Tongue
English
Weight
462 KB
Volume
22
Category
Article
ISSN
0733-2459

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✦ Synopsis


we performed two consecutive, prospective trials studying corticosteroids and cyclosporine (CSA) as adjuncts to plasma exchange (PE). Methods: Concurrent Corticosteroids and PE: Prednisone (1 mg/kg/d) was given concurrent with PE and continued until remission was achieved. Remission was defined as a normal platelet count and normalization of the lactate dehydrogenase (LDH). Prednisone was tapered over 4 weeks following remission. Concurrent CSA and PE: Oral CSA at a dose of 2-3 mg/kg/day in a divided dose was started concurrently with PE therapy and continued for 6 months. After analyzing data from our previous study of CSA in recurrent TTP, patients were given the option to continue CSA beyond the planned 6 months. Results: Concurrent Corticosteroids and PE: Ten of 12 patients (83%) achieved remission after a median of 6.5 exchanges (range, 4-10). Six of 10 (60%) patients suffered exacerbations, defined as the need to reinitiate PE therapy within 30 days of the last exchange. Two patients relapsed after 5 and 8 months; 2 have maintained a continuous remission for 38 and 30 months. Concurrent CSA and PE: Ten of 11 (91%) patients achieved remission after a median of five exchanges (range, 3-9), with 9 patients evaluable for follow-up. No patients suffered an exacerbation; two relapsed during the 6-month course of CSA, with one patient relapsing 2 weeks after a 50% dose reduction for renal insufficiency. Seven of 9 patients remained in continuous remission after completing 6 months of CSA. Three patients stopped CSA and 4 continued CSA beyond 6 months. Of the 3 stopping CSA, one relapsed 2 weeks after discontinuing therapy, with 2 continuing in remission 12 and 13 months after stopping CSA. Serial measurements of ADAMTS13 activity, inhibitor concentration, and antigen during CSA therapy are shown in the figure later. Conclusions: CSA appears to be superior to corticosteroids as an adjunct to PE in the initial treatment of TTP based upon a significant reduction in the exacerbation rate. CSA also appeared to improve ADAMTS13 activity via the suppression of the inhibitor of ADAMTS13.


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