Low- and intermediate-risk myelodysplastic syndrome with pure red cell aplasia
Low- and intermediate-risk myelodysplastic syndrome with pure red cell aplasia
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DOI：
10.1080/16078454.2021.1929694
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发表时间：
2021-01-01
期刊：
影响因子：
1.9
通讯作者：
Fu, Rong
中科院分区：
文献类型：
--
作者：
Wang, Huaquan;Niu, Haiyue;Fu, Rong
目的：探讨中低危骨髓增生异常综合征(MDS)合并纯红细胞再生障碍性贫血(PRCA)的临床特点。方法：回顾分析2010年1月至2019年12月本院确诊的中低危MDS合并PRCA患者的临床特点。占同期诊断的PRCA和MDS的7.7%(6/78)和1.67%(6/359)。所有患者均接受多种药物治疗，包括重组人促红细胞生成素、环孢素、糖皮质激素、雄激素、西罗莫司、静脉注射免疫球蛋白和地西他滨。2例患者完全缓解，2例患者部分缓解，实现输血独立。2例无反应，1例死亡。结论：中低危MDS合并PRCA治疗困难，但预后良好。
Objectives: Our aim is to investigate the clinical characteristics of low- and intermediate-risk myelodysplastic syndrome (MDS) with pure red cell aplasia (PRCA).Methods: We retrospectively reviewed the patients of low- and intermediate-risk MDS patients who had been diagnosed with PRCA in our hospital between January 2010 and December 2019.Results: There were 6 low- and intermediate-risk MDS patients with PRCA in our study, 1 male and 5 females, with a median age of 63.5 (50-75) years. It accounted for 7.7% (6/78) of all diagnosed PRCA cases and 1.67% (6/359) of diagnosed MDS cases during the same period. All patients were treated with multiple drugs, including recombinant human erythropoietin, cyclosporine, glucocorticoids, androgen, sirolimus, intravenous immunoglobulin and decitabine. Two patients achieved complete remission, two patients achieved partial remission and became blood transfusion independent. Two patients had no response and one patient died.Conclusion: Low- and intermediate-risk MDS with PRCA was difficult to treat, but the prognosis was good.