XXXX ( @entity2106 ): initial treatment with plasma exchange plus @entity230 and immunosuppressive agents for relapsing cases.

@entity1 @entity230 @entity8 @entity1396 @entity55 @entity16 @entity1252 @entity786 @entity134 @entity1891 @entity1011 @entity395 @entity221 @entity2106
BACKGROUND: The @entity2106 ( @entity2106 ) is a @entity395 with a high mortality rate if untreated or delayed therapy. Whether immediate diagnosis and promptly treated with plasma exchange can change the grave prognosis? METHOD: Retrospective analysis was performed on clinical characteristics and treatment outcome of 13 @entity1 diagnosed and treated during a 6-year period, from August 1994 to August 2000, in a tertiary care university hospital in Taiwan. RESULTS: Among the @entity1 , 8 were males and 5 were females, 10 were idiopathic, 2 were @entity1396 , and 1 was @entity1252 -induced; 12 (92.3%) had @entity16 , 11 (84.6%) had ecchymosis, 8 (61.5%) had @entity221 , and 6 (46.2%) had @entity8 ( @entity1011 > or = 1.5 mg/dL) at initial presentation of the syndrome. Excluding the @entity1252 @entity1 , 6 of 10 (60%) had shown antinuclear antibody (ANA) non-specific positive (titer > or = 1:40). All @entity1 were initially treated with plasma exchange plus @entity230 . Of these 13 @entity1 , 11 (84.6%) achieved complete remission, one had partial remission, and one, which was @entity1396 -induced, had no response and died of a progressive disease complicated with @entity55 . Within a median follow-up period of 31 months, 4 of 11 @entity1 who achieved complete remission relapsed after one week, two weeks, three weeks, and three months, respectively. In the four relapsing @entity1 , three late relapsing @entity1 received FFP infusion, increased @entity134 dosages, added @entity786 plus @entity1891 ; and one early relapsing @entity1 , relapsing twice, received an additional two courses of plasma exchange and added @entity786 plus @entity1891 . All of the four @entity1 achieved complete remission again. The @entity1 who had partial remission relapsed early and responded promptly to another course of plasma exchange plus @entity786 and @entity1891 and achieved complete remission. CONCLUSION: Based on the results in this study, we conclude that plasma exchange plus @entity230 can effectively treat @entity2106 . For @entity1 with a refractory or relapsing disease, immunosuppressive therapy with @entity786 plus @entity1891 should be administered as well.
