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
No coin nor oath required. For personal study only.
✦ 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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