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一名患有类风湿关节炎的老年男性获得性因子VIII缺乏症的罕见病例。

A Rare Case of Acquired Factor VIII Deficiency in an Elderly Male With a History of Rheumatoid Arthritis.

作者信息

Shah Shubhangi, Tseng Michael, Durojaiye Ashimiyu

机构信息

Internal Medicine, Virginia Commonwealth University School of Medicine, Richmond, USA.

Hematology and Medical Oncology, Virginia Commonwealth University School of Medicine, Richmond, USA.

出版信息

Cureus. 2023 Aug 26;15(8):e44169. doi: 10.7759/cureus.44169. eCollection 2023 Aug.

DOI:10.7759/cureus.44169
PMID:37753049
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10519439/
Abstract

Acquired hemophilia A (AHA) or factor VIII (FVIII) deficiency is caused by autoantibodies targeting FVIII in the blood coagulation pathway; it is a rare condition making it challenging to diagnose. A timely diagnosis is crucial, without which there is a risk of catastrophic bleeding. We report a case of a patient with a history of duodenal arteriovenous malformations, previously on apixaban, who presented with four days of melena. On admission he was found to have a hemoglobin of 5.7 and elevated partial thromboplastin time (PTT), promoting further workup showing FVIII levels of <1%, with a mixing study that failed to correct suggesting the presence of inhibitors against FVIII. Other characteristics of this patient's cases included controlled rheumatoid arthritis without detectable rheumatoid factor or increased erythrocyte sedimentation rate (ESR). The patient was initially treated with prednisone and intravenous immunoglobulins, but an insufficient response prompted the initiation of recombinant factor VII, rituximab, and cyclophosphamide during hospitalization.

摘要

获得性血友病A(AHA)或因子VIII(FVIII)缺乏症是由针对凝血途径中FVIII的自身抗体引起的;这是一种罕见疾病,诊断具有挑战性。及时诊断至关重要,否则有发生灾难性出血的风险。我们报告一例有十二指肠动静脉畸形病史、之前服用阿哌沙班的患者,该患者出现了四天的黑便。入院时发现他的血红蛋白为5.7,部分凝血活酶时间(PTT)升高,进一步检查显示FVIII水平<1%,混合试验未能纠正,提示存在针对FVIII的抑制剂。该患者病例的其他特征包括类风湿关节炎得到控制,类风湿因子未检测到,红细胞沉降率(ESR)未升高。患者最初接受泼尼松和静脉注射免疫球蛋白治疗,但反应不足促使在住院期间开始使用重组因子VII、利妥昔单抗和环磷酰胺。

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本文引用的文献

1
A Case Report of Idiopathic Acquired Hemophilia Type A.一例获得性甲型特发性血友病病例报告。
Cureus. 2023 May 6;15(5):e38634. doi: 10.7759/cureus.38634. eCollection 2023 May.
2
Acquired hemophilia A following COVID-19 vaccine: a case report.COVID-19 疫苗接种后获得性血友病 A:病例报告。
J Med Case Rep. 2023 Mar 27;17(1):125. doi: 10.1186/s13256-023-03850-z.
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Acquired Hemophilia A.获得性血友病 A。
Hematol Oncol Clin North Am. 2021 Dec;35(6):1131-1142. doi: 10.1016/j.hoc.2021.07.007. Epub 2021 Sep 15.
4
A Case of Acquired Hemophilia A Following SARS-CoV-2 Infection.1例新型冠状病毒感染后获得性甲型血友病病例。
Cureus. 2021 Jul 23;13(7):e16579. doi: 10.7759/cureus.16579. eCollection 2021 Jul.
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Surgery-associated acquired hemophilia A: a report of 2 cases and review of literature.手术相关获得性血友病 A:2 例报告并文献复习。
BMC Surg. 2020 Sep 23;20(1):213. doi: 10.1186/s12893-020-00872-y.
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Bullous Pemphigoid Associated with Acquired Hemophilia A: A Case Report and Review of the Literature.大疱性类天疱疮合并获得性血友病A:一例报告及文献复习
Case Rep Dermatol. 2019 May 21;11(2):130-139. doi: 10.1159/000499525. eCollection 2019 May-Aug.
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Acquired hemophilia A presenting as progressive intra-abdominal hemorrhage, muscle hemorrhage and hemothorax postpartum: A case report and literature review.产后以进行性腹腔内出血、肌肉出血和血胸为表现的获得性甲型血友病:一例报告及文献复习
Exp Ther Med. 2019 Jan;17(1):633-638. doi: 10.3892/etm.2018.7031. Epub 2018 Nov 29.
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Acquired Hemophilia Secondary to Soft-tissue Sarcoma: Case Report from a Latin American Hospital and Literature Review.软组织肉瘤继发获得性血友病:来自一家拉丁美洲医院的病例报告及文献综述
Cureus. 2018 May 14;10(5):e2621. doi: 10.7759/cureus.2621.
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Targeting Acquired Hemophilia A with Rheumatoid Arthritis by a Rituximab Shot: A Case Report and Review of the Literature.利妥昔单抗注射治疗合并类风湿关节炎的获得性血友病A:1例病例报告及文献复习
Am J Case Rep. 2018 May 21;19:582-588. doi: 10.12659/AJCR.908854.
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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。
Am J Hematol. 2017 Jul;92(7):695-705. doi: 10.1002/ajh.24777. Epub 2017 Jun 5.