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利妥昔单抗与环磷酰胺治疗慢性淋巴细胞白血病合并获得性血友病A耐药病例的安全性与疗效

Safety and Efficacy of Rituximab and Cyclophosphamide in a Case of Resistant Acquired Hemophilia A in Course of Chronic Lymphocytic Leukemia.

作者信息

Mazziotta Francesco, Cecconi Nadia, Iovino Lorenzo, Cervetti Giulia, Petrini Mario

机构信息

Genomec School of Doctorate, University of Siena, Siena, ITA.

Clinical and Experimental Medicine, University of Pisa, Pisa, ITA.

出版信息

Cureus. 2019 Sep 12;11(9):e5630. doi: 10.7759/cureus.5630.

DOI:10.7759/cureus.5630
PMID:31700733
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6822901/
Abstract

Acquired Hemophilia A (AHA) is a rare disease caused by anti-factor VIII autoantibodies. It is usually characterized by clinically significant bleeding at the onset and requires prompt hemostatic and immunosuppressive therapies. Due to its rarity and the lack of randomized trials, its treatment is a great challenge, especially in the relapse/refractory setting. This report presents the case of a patient diagnosed with chronic lymphocytic leukemia who developed a steroid-resistant AHA, successfully managed with aggressive immunosuppressive therapy.

摘要

获得性血友病A(AHA)是一种由抗凝血因子VIII自身抗体引起的罕见疾病。其通常的特征是发病时出现具有临床意义的出血,需要迅速进行止血和免疫抑制治疗。由于其罕见性以及缺乏随机试验,其治疗是一项巨大挑战,尤其是在复发/难治性情况下。本报告介绍了一例被诊断为慢性淋巴细胞白血病且发生了类固醇抵抗性AHA的患者,该患者通过积极的免疫抑制治疗成功得到了处理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e00f/6822901/d4f97d7e6854/cureus-0011-00000005630-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e00f/6822901/d4f97d7e6854/cureus-0011-00000005630-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e00f/6822901/d4f97d7e6854/cureus-0011-00000005630-i01.jpg

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本文引用的文献

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Chronic lymphocytic leukemia: 2017 update on diagnosis, risk stratification, and treatment.慢性淋巴细胞白血病:2017 年诊断、风险分层和治疗更新。
Am J Hematol. 2017 Sep;92(9):946-965. doi: 10.1002/ajh.24826.
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Acquired hemophilia A: Updated review of evidence and treatment guidance.获得性血友病 A:证据和治疗指南的最新综述。
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Acquired factor VIII inhibitor and subsequent development of non-Hodgkin's lymphoma: a case report and review of the literature.
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Blood. 2012 Jul 5;120(1):47-55. doi: 10.1182/blood-2012-02-409185. Epub 2012 Apr 18.
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Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2).获得性血友病 A 的人口统计学和临床数据:来自欧洲获得性血友病登记处(EACH2)的结果。
J Thromb Haemost. 2012 Apr;10(4):622-31. doi: 10.1111/j.1538-7836.2012.04654.x.
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Leukemia. 2011 Mar;25(3):473-8. doi: 10.1038/leu.2010.278. Epub 2010 Dec 3.
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