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环孢素A与皮质类固醇联合使用可使获得性纯红细胞再生障碍性贫血成年患者更快地诱导完全缓解。

Induced complete remission faster in adult patients with acquired pure red cell aplasia by combining cyclosporine A with corticosteroids.

作者信息

Wu Xuemei, Yang Yan, Lu Xingyu, Yin Hua, Wang Suli, Wang Shuai, Hong Ming, Zhu Yu, Lu Ruinan, Qiao Chun, Wu Yujie, He Guangsheng, Li Jianyong

机构信息

Department of Hematology, Jiangsu Province Hospital, The First Affiliated Hospital of Nanjing Medical University, Key Laboratory of Hematology of Nanjing Medical University, Collaborative Innovation Center for Cancer Personalized Medicine Nanjing, Jiangsu Province.

First Affiliated Hospital of Jilin University, Changchun, Jilin Province, China.

出版信息

Medicine (Baltimore). 2019 Oct;98(41):e17425. doi: 10.1097/MD.0000000000017425.

DOI:10.1097/MD.0000000000017425
PMID:31593095
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6799648/
Abstract

To evaluate whether the adult patients with acquired pure red cell aplasia (PRCA) could benefit more from cyclosporine A (CsA) combined with corticosteroids (CS) than CsA or CS alone.Seventy-three patients were evaluated in 2 institutions (6 patients lost to follow-up).The induction therapy included CsA (n = 21), CS (n = 21), or CsA combined with CS (n = 31), and remission was achieved in 16/21 (76.2%), 10/21 (47.6%), and 21/31 (71.0%) patients, respectively. Higher complete remission (CR) rate was achieved in CsA combined with CS group than in CS group (61.3% vs 19.0%, P = .003). Patients achieved CR faster in CsA combined with CS group than in CS group or CsA group (median time, 1 month vs 2 month vs 3 month, P = .010). By multivariate analysis, CsA combined with CS therapy and primary PRCA were the influence factors for CR rate. Twenty-seven patients relapsed due to discontinuation or tapering therapy, and 19 patients regained response by increasing the dose of original regimens or changing to other immunosuppressive therapy. Complete remission to induction therapy was a correlative factor for death (P = .035).CsA combined with CS produced faster and higher CR rate in treating adult patients with PRCA than did CsA or CS alone.

摘要

评估获得性纯红细胞再生障碍性贫血(PRCA)成年患者使用环孢素A(CsA)联合皮质类固醇(CS)治疗是否比单独使用CsA或CS更有益。在2家机构对73例患者进行了评估(6例患者失访)。诱导治疗包括CsA(n = 21)、CS(n = 21)或CsA联合CS(n = 31),分别有16/21(76.2%)、10/21(47.6%)和21/31(71.0%)的患者实现缓解。CsA联合CS组的完全缓解(CR)率高于CS组(61.3%对19.0%,P = 0.003)。CsA联合CS组患者达到CR的速度比CS组或CsA组更快(中位时间,1个月对2个月对3个月,P = 0.010)。多因素分析显示,CsA联合CS治疗和原发性PRCA是CR率的影响因素。27例患者因停药或减药治疗而复发,19例患者通过增加原方案剂量或改用其他免疫抑制治疗恢复缓解。诱导治疗的完全缓解是死亡的相关因素(P = 0.035)。与单独使用CsA或CS相比,CsA联合CS治疗成年PRCA患者能更快获得更高的CR率。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a869/6799648/ecde6341f588/medi-98-e17425-g008.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a869/6799648/8891ab247480/medi-98-e17425-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a869/6799648/12c0839f3fa6/medi-98-e17425-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a869/6799648/ecde6341f588/medi-98-e17425-g008.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a869/6799648/8891ab247480/medi-98-e17425-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a869/6799648/12c0839f3fa6/medi-98-e17425-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a869/6799648/ecde6341f588/medi-98-e17425-g008.jpg

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本文引用的文献

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Int J Hematol. 2018 Aug;108(2):123-129. doi: 10.1007/s12185-018-2446-y. Epub 2018 Mar 27.
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Pure red cell aplasia.纯红细胞再生障碍。
Blood. 2016 Nov 24;128(21):2504-2509. doi: 10.1182/blood-2016-05-717140.
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Guidelines for the diagnosis and management of adult aplastic anaemia.成人再生障碍性贫血诊断与治疗指南。
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Long-term outcome of patients with acquired chronic pure red cell aplasia (PRCA) following immunosuppressive therapy: a final report of the nationwide cohort study in 2004/2006 by the Japan PRCA collaborative study group.2004/2006 年日本获得性慢性纯红细胞再生障碍性贫血合作研究组全国性队列研究:免疫抑制治疗后获得性慢性纯红细胞再生障碍性贫血患者的长期预后:最终报告。
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CDR3-independent expansion of Vδ1 T lymphocytes in acquired chronic pure red cell aplasia.获得性慢性纯红细胞再生障碍性贫血中 Vδ1 T 淋巴细胞的 CDR3 非依赖性扩增。
Immunol Lett. 2013 Feb;150(1-2):23-9. doi: 10.1016/j.imlet.2012.12.006. Epub 2012 Dec 27.
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Expansion of CD8+/perforin+ T-cells predicts response to ciclosporin A therapy in patients with erythroid hypoplasia/aplasia.
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Expansion of CD8+ /perforin+ effector memory T cells in the bone marrow of patients with thymoma-associated pure red cell aplasia.胸腺瘤相关纯红细胞再生障碍患者骨髓中CD8 + /穿孔素+效应记忆T细胞的扩增。
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