A retrospective analysis of 84 adult patients with Evans syndrome in a single center / 中华血液学杂志

ABSTRACT

<p><b>OBJECTIVE</b>To evaluate the initial clinical characteristics, the response to treatment, and the outcome in adult patients with Evans syndrome.</p><p><b>METHODS</b>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.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSIONS</b>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.</p>