[A retrospective analysis of 84 adult patients with Evans syndrome in a single center].
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.
Institute of Hematology & Blood Diseases Hospital, CAMS & PUMC, Tianjin 300020, China.
Xun-Wei
XW
Dong
Li-Peng
LP
Zhang
Yu-Ping
YP
Zhao
Yi-Zhou
YZ
Zheng
Feng-Kui
FK
Zhang
Ren-Chi
RC
Yang
D000305:Adrenal Cortex Hormones; D000328:Adult; D000744:Anemia, Hemolytic, Autoimmune; D005500:Follow-Up Studies; D006801:Humans; D012074:Remission Induction; D012189:Retrospective Studies; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones