CAJ | 학술논문

目的探讨成人Evans综合征的初始临床特征,各种治疗方法的疗效及病程.方法对84例成人Evans综合征患者(男20例,女64例)应用激素、丙种免疫球蛋白、免疫抑制剂多药联合治疗,中位随访17.5(0.03～140)个月,观察患者起病特征及临床疗效.结果所有患者均接受激素加或不加静脉丙种免疫球蛋白初始治疗.47例患者单用激素治疗,其中38例达完全缓解(CR)和部分缓解(PR).中位随访12个月,92.1%的患者复发.28例对激素耐药或出现严重出血的患者随后给予免疫抑制剂治疗,89.3%的患者获得CR或PR.中位随访8个月,84%患者复发.结论 Evans 综合征难治且易复发,联合治疗可能是治疗Evans综合征的有效手段.
목적 탐토성인Evans종합정적초시림상특정,각충치료방법 적료효급병정.방법 대84례성인Evans종합정환자(남20례,녀64례)응용격소、병충면역구단백、면역억제제다약연합치료,중위수방17.5(0.03～140)개월,관찰환자기병특정급림상료효.결과 소유환자균접수격소가혹불가정맥병충면역구단백초시치료.47례환자단용격소치료,기중38례체완전완해(CR)화부분완해(PR).중위수방12개월,92.1%적환자복발.28례대격소내약혹출현엄중출혈적환자수후급여면역억제제치료,89.3%적환자획득CR혹PR.중위수방8개월,84%환자복발.결론 Evans 종합정난치차역복발,연합치료가능시치료Evans종합정적유효수단.
Objective To evaluate the initial clinical characteristics, the response to treatment, and the outcome in adult patients with Evans syndrome. Methods The clinical data of 84 adult patients (20 males, 64 females) with Evans syndrome diagnosed at our center between 1984 and 2007 were retrospectively analyzed. Results The patients were followed up for a median duration of 17.5 (0.03-140) months. All the patients initially received intravenous steroids with or without intravenous immunoglobulin (IVIG). Forty-seven patients were treated with corticosteroids alone initially. Complete remission (CR) and partial remission (PR) were achieved in 38 of the patients, but 92. 1% of them relapsed during a median follow-up of 12 months. Twenty-eight patients who were resistant to corticosteroids therapy or with severe bleeding were subsequently administered immunosupressive agents. CR and PR were obtained in 89.3% of them. Within a median follow-up of 8 months, 84% of these patients relapsed. Conclusions Evans syndrome is a chronic and easy to recurrent disease, which is often refractory to conventional therapy. Treatment with combination agents might be a useful therapeutic approach to the patients.