Background: In adults with immune thrombocytopenic purpura (ITP , Corticosteroids (steroid)remains the initial therapy of choice. However, a significant proportion of these patients become dependant on it for maintaining remission or become refractory to it. The long-term outcome of splenectomy in these patients (pts) continues to be defined and current information on the management of post-splenectomy relapses is inadequate. Methods: A retrospective review of the medical records of consecutive adu t pts with ITP, who underwent splenectomy at the Mayo Clinic between 1985 and 199 for steroid-dependent or refractory disease. Pts with underlying lymphoproliferativ : disorders were excluded. Updated information was obtained through patient or physicia i contact. Results: During the study period, 147 pts (median age, 51 years; range, 16-88; 87 females) underwent splenectomy for steroid -refractory (63 pts) or steroid-depender t ITP (74 pts). At initial diagnosis of ITP, 138 pts (94%) were treated with steroids with a i approximate 80% overall response rate. The median time to splenectomy was 8 montl i (range, 1 month to 26 years). At 30 days post splenectomy, 110 pts (75%) had a complet ! remission (CR = platelet count 150 x 109/L), 21 (14%) had a partial remission (PR ;= platelet count 50-150 x 109/L) and 16(11%) had a transient or no response. At one-yea r post splenectomy, 82 pts (56%) were in CR, 7 (5%) in PR, 23 (16%) in relapse, and no follow-up information was available for the remaining 35 (24%). Pts with an early responsi : to splenectomy were younger (p=0.003). In a multivariate analysis including several pns and post splenectomy variables, steroid refractory patients were more likely to relapse after an initial CR (RR 6.2). Of the 82 pts who were in CR at one year, only four relapse< 1 subsequently. Seventy-four episodes of post-splenectomy relapse were treated wit] i prednisone (17/23 responses), danazol with or without prednisone (23/25 responses) vincristine (3/6 responses), and rituxan (1/1 response), and various other agents (al transient responses). There were three deaths related to postoperative complications ant one due to post splenectomy sepsis. Conclusion: Splenectomy offers potential long term remission for adults with ITP regardless of whether they were steroid-dependent or steroid-refractory before the procedure. Most of the post-splenectomy relapses occur ir the initial three months and the relapse rate is very low after the first year. Post-splenectom; relapses can be effectively treated by various agents including danazol, steroids, anc vincristine. \.
|Original language||English (US)|
|Issue number||11 PART I|
|State||Published - Dec 1 2000|
ASJC Scopus subject areas
- Cell Biology