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低危和中危骨髓增生异常综合征伴纯红细胞再生障碍。

Low- and intermediate-risk myelodysplastic syndrome with pure red cell aplasia.

机构信息

Department of Hematology, General Hospital, Tianjin Medical University, Tianjin, People's Republic of China.

出版信息

Hematology. 2021 Dec;26(1):444-446. doi: 10.1080/16078454.2021.1929694.

Abstract

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.

摘要

目的

本研究旨在探讨伴有纯红细胞再生障碍性贫血(PRCA)的低危和中危骨髓增生异常综合征(MDS)的临床特征。

方法

我们回顾性分析了 2010 年 1 月至 2019 年 12 月期间在我院诊断为 PRCA 的低危和中危 MDS 患者。

结果

本研究共纳入 6 例低危和中危 MDS 伴 PRCA 患者,男 1 例,女 5 例,中位年龄 63.5(50-75)岁。该组病例占同期所有 PRCA 诊断病例的 7.7%(6/78),同期所有 MDS 诊断病例的 1.67%(6/359)。所有患者均接受了多种药物治疗,包括重组人红细胞生成素、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 例患者达到完全缓解,2 例患者达到部分缓解且不再依赖输血,2 例患者无反应,1 例患者死亡。

结论

低危和中危 MDS 伴 PRCA 治疗困难,但预后良好。

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