成功通过免疫吸附治疗血友病A伴VIII因子抑制物患者的危及生命的出血，但抗CD20治疗未实现长期缓解。
Successful immunoadsorption of life-threatening bleeding in factor VIII inhibitor disease, but no long-term remission with anti-CD20 treatment.
作者信息
Grahammer Florian, Fischer Karl-Georg
机构信息
Renal Division, University Medical Center Freiburg, Freiburg, Germany.
出版信息
BMJ Case Rep. 2015 Aug 30;2015:bcr2015210034. doi: 10.1136/bcr-2015-210034.
Abstract
A 62-year-old man and a 64-year-old woman presented to our institution with acquired haemophilia A. They both developed life-threatening bleeding. Immunoadsorption using protein A columns was used to rapidly lower factor VIII inhibitor levels. Immunosuppression with steroids and the anti-CD20 antibody, rituximab, was instituted. Yet their effects were either partial or complicated by an early relapse. Repetitive cyclophosphamide administration led to a sustained immunological response. While immunoadsorption appears effective and safe to lower factor VIII inhibitor levels, it seems that further preferably randomised controlled trials are needed to establish the value of rituximab versus the standard immunosuppressive regime comprising cyclophosphamide.
摘要
一名62岁男性和一名64岁女性因获得性血友病A前来我院就诊。他们均出现了危及生命的出血情况。使用蛋白A柱进行免疫吸附以迅速降低凝血因子VIII抑制物水平。采用类固醇和抗CD20抗体利妥昔单抗进行免疫抑制治疗。然而，其效果要么是部分有效，要么因早期复发而变得复杂。重复给予环磷酰胺导致了持续的免疫反应。虽然免疫吸附在降低凝血因子VIII抑制物水平方面似乎有效且安全，但似乎需要进一步开展更理想的随机对照试验，以确定利妥昔单抗相对于包含环磷酰胺的标准免疫抑制方案的价值。
相似文献
1
Successful immunoadsorption of life-threatening bleeding in factor VIII inhibitor disease, but no long-term remission with anti-CD20 treatment.成功通过免疫吸附治疗血友病A伴VIII因子抑制物患者的危及生命的出血，但抗CD20治疗未实现长期缓解。
BMJ Case Rep. 2015 Aug 30;2015:bcr2015210034. doi: 10.1136/bcr-2015-210034.
2
Acquired hemophilia in the patient suffering from rheumatoid arthritis: case report.类风湿关节炎患者获得性血友病：病例报告
Blood Coagul Fibrinolysis. 2013 Dec;24(8):874-80. doi: 10.1097/MBC.0b013e3283646635.
3
[Long-term response to rituximab in a patient with acquired hemophilia].[利妥昔单抗对一名获得性血友病患者的长期疗效]
Rev Invest Clin. 2011 Mar-Apr;63(2):210-2.
4
Rituximab for the treatment of patients with very high-titre acquired factor VIII inhibitors refractory to conventional chemotherapy.利妥昔单抗用于治疗对传统化疗难治的高滴度获得性凝血因子VIII抑制物患者。
Haemophilia. 2007 Jan;13(1):46-50. doi: 10.1111/j.1365-2516.2006.01342.x.
5
Immunosuppression for acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2).获得性血友病 A 的免疫抑制治疗：来自欧洲获得性血友病登记处（EACH2）的结果。
Blood. 2012 Jul 5;120(1):47-55. doi: 10.1182/blood-2012-02-409185. Epub 2012 Apr 18.
6
Acquired haemophilia A associated with autoimmune thyroiditis: a case report.获得性血友病A合并自身免疫性甲状腺炎：一例报告
J Med Case Rep. 2014 Dec 29;8:469. doi: 10.1186/1752-1947-8-469.
7
Treatment of minor severe acquired haemophilia. Is there a rationale for immunoadsorption?轻度严重获得性血友病的治疗。免疫吸附疗法是否有理论依据？
Atheroscler Suppl. 2015 May;18:74-9. doi: 10.1016/j.atherosclerosissup.2015.02.015.
8
Acquired haemophilia syndrome: pathophysiology and therapy.获得性血友病综合征：病理生理学与治疗
Srp Arh Celok Lek. 2010 Jan;138 Suppl 1:64-8. doi: 10.2298/sarh10s1064e.
9
Combination therapy for inhibitor reversal in haemophilia A using monoclonal anti-CD20 and rapamycin.使用单克隆抗CD20和雷帕霉素联合治疗血友病A中的抑制剂逆转
Thromb Haemost. 2017 Jan 5;117(1):33-43. doi: 10.1160/TH16-05-0404. Epub 2016 Sep 29.
10
Acquired haemophilia A. First line treatment with calcineurin inhibitors and steroid pulses: a 10-year follow-up study.获得性血友病 A. 一线治疗采用钙调神经磷酸酶抑制剂和类固醇脉冲：一项 10 年随访研究。
Haemophilia. 2012 Sep;18(5):789-93. doi: 10.1111/j.1365-2516.2012.02772.x. Epub 2012 Mar 19.
引用本文的文献
1
Acquired haemophilia A: Italian Consensus Recommendations on diagnosis, general management and treatment of bleeding.获得性血友病 A：意大利共识建议关于出血的诊断、一般管理和治疗。
Blood Transfus. 2022 May;20(3):245-262. doi: 10.2450/2022.0238-21. Epub 2022 Jan 20.
2
Rituximab for eradicating inhibitors in people with acquired haemophilia A.利妥昔单抗治疗获得性血友病 A 患者的抑制剂
Cochrane Database Syst Rev. 2021 Aug 23;8(8):CD011907. doi: 10.1002/14651858.CD011907.pub3.
3
Rituximab for eradicating inhibitors in people with acquired haemophilia A.利妥昔单抗用于清除获得性甲型血友病患者体内的抑制物。
Cochrane Database Syst Rev. 2016 Jul 8;7(7):CD011907. doi: 10.1002/14651858.CD011907.pub2.
本文引用的文献
1
Immunosuppression for acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2).获得性血友病 A 的免疫抑制治疗：来自欧洲获得性血友病登记处（EACH2）的结果。
Blood. 2012 Jul 5;120(1):47-55. doi: 10.1182/blood-2012-02-409185. Epub 2012 Apr 18.
2
The relevance of the bleeding severity in the treatment of acquired haemophilia - an update of a single-centre experience with 67 patients.获得性血友病治疗中出血严重程度的相关性 - 单中心 67 例经验更新。
Haemophilia. 2010 May;16(102):95-101. doi: 10.1111/j.1365-2516.2008.01922.x. Epub 2008 Nov 14.
3
Rituximab in the treatment of adult acquired hemophilia A: a systematic review.
Crit Rev Oncol Hematol. 2007 Jul;63(1):47-52. doi: 10.1016/j.critrevonc.2006.11.004. Epub 2007 Jan 19.
4
Acquired hemophilia A: a concise review.获得性血友病A：简要综述。
Am J Hematol. 2005 Sep;80(1):55-63. doi: 10.1002/ajh.20390.
5
Treatment of acquired hemophilia by the Bonn-Malmo Protocol: documentation of an in vivo immunomodulating concept.采用波恩-马尔默方案治疗获得性血友病：一种体内免疫调节概念的记录
Blood. 2005 Mar 15;105(6):2287-93. doi: 10.1182/blood-2004-05-1811. Epub 2004 Nov 12.
6
Selective B-cell depletion with rituximab for the treatment of patients with acquired hemophilia.使用利妥昔单抗进行选择性B细胞清除治疗获得性血友病患者。
Blood. 2004 Jun 15;103(12):4424-8. doi: 10.1182/blood-2003-11-4075. Epub 2004 Mar 2.
7
Immunoadsorption may provide a cost-effective approach to management of patients with inhibitors to FVIII.
Transfusion. 2003 Nov;43(11):1508-13. doi: 10.1046/j.1537-2995.2003.00559.x.
8
Acquired high-titer factor VIII inhibitor: fatal bleeding despite multimodal treatment including rituximab preceded by multiple plasmaphereses.
Blood. 2003 May 1;101(9):3753-4; author reply 3754-5. doi: 10.1182/blood-2002-12-3920.
9
Rituximab in the treatment of acquired factor VIII inhibitors.
Blood. 2002 Nov 1;100(9):3426-8. doi: 10.1182/blood-2002-03-0765.
10
Treatment of coagulation inhibitors with extracorporeal immunoadsorption (Ig-Therasorb).
Br J Haematol. 2001 Jan;112(1):91-7. doi: 10.1046/j.1365-2141.2001.02510.x.
Zotero 插件