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病例报告:采用 CyDRI 方案有效治疗阿达木单抗诱导的获得性血友病 A

Case Report: Effective management of adalimumab-induced acquired hemophilia A with the CyDRI protocol.

机构信息

Departments of Hematology and Stem Cell Transplantation, South Pest Central Hospital, National Institute of Hematology and Infectious Diseases, Budapest, Hungary.

Department of Public Health, Semmelweis University, Budapest, Hungary.

出版信息

Pathol Oncol Res. 2024 May 23;30:1611720. doi: 10.3389/pore.2024.1611720. eCollection 2024.

Abstract

INTRODUCTION

Acquired Hemophilia A (AHA) is a rare autoimmune disorder characterized by the emergence of inhibitors that specifically target coagulation Factor VIII, frequently resulting in severe bleeding episodes.

METHODS

We conducted a retrospective analysis of the medical records of a 68-year-old male patient who presented with adalimumab-induced AHA.

RESULTS

The patient received adalimumab, a tumor necrosis factor inhibitor antibody, as part of his treatment for rheumatoid arthritis. The patient's clinical journey, characterized by intense bleeding and coagulopathy, was effectively managed with the application of recombinant Factor VIIa (rFVIIa) and the CyDRi protocol.

DISCUSSION

The case emphasizes the importance of prompt coagulation assessment in patients with bleeding symptoms receiving disease-modifying therapy for rheumatoid arthritis that includes adalimumab therapy, considering the rare yet life-threatening nature of AHA. Additionally, this report provides an extensive review of the existing literature on drug-induced AHA, with a special emphasis on cases linked to immunomodulatory medications. Through this two-pronged approach, our report aims to enhance understanding and awareness of this severe complication among healthcare providers, promoting timely diagnosis and intervention.

摘要

简介

获得性血友病 A(AHA)是一种罕见的自身免疫性疾病,其特征是出现专门针对凝血因子 VIII 的抑制剂,经常导致严重的出血事件。

方法

我们对一名 68 岁男性患者的病历进行了回顾性分析,该患者患有阿达木单抗诱导的 AHA。

结果

该患者接受阿达木单抗治疗,阿达木单抗是一种肿瘤坏死因子抑制剂抗体,作为类风湿关节炎治疗的一部分。该患者的临床病程以严重出血和凝血功能障碍为特征,通过应用重组 VIIa 因子(rFVIIa)和 CyDRi 方案得到了有效管理。

讨论

该病例强调了在接受包括阿达木单抗治疗在内的类风湿关节炎疾病修正治疗的患者中,出现出血症状时及时进行凝血评估的重要性,因为 AHA 虽然罕见但具有致命性。此外,本报告对药物诱导的 AHA 现有文献进行了广泛综述,特别强调了与免疫调节药物相关的病例。通过这两方面的方法,我们的报告旨在提高医疗保健提供者对这种严重并发症的认识和意识,促进及时诊断和干预。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8e1c/11153699/200c7c7d6af5/pore-30-1611720-g001.jpg

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