获得性血友病A的诊断和治疗:病例报告和文献综述。

IF 0.8 Q3 MEDICINE, GENERAL & INTERNAL Case Reports in Medicine Pub Date : 2021-09-14 eCollection Date: 2021-01-01 DOI:10.1155/2021/5554664
Ikhwan Rinaldi, Findy Prasetyawaty, Siti Fazlines, Kevin Winston, Yusuf Aji Samudera Nurrobi, Jessica Leoni, Ilham Hidayat Restu Tulus Maha, Satrio Wicaksono, Abdillah Yasir Wicaksono, Averina Octaxena Aslani, Rizkania Ikhsani
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引用次数: 1

摘要

背景:获得性血友病A (AHA)是一种潜在危及生命的自身免疫性止血疾病,其血液循环中存在破坏因子VIII (FVIII)功能的自身抗体。AHA的早期诊断是困难的,因为AHA的症状不同于先天性a型血友病。此外,由于存在针对FVIII (FVIII抑制剂)的自身抗体,AHA的治疗也更为复杂。在这里,我们提出三个病例报告,并进行文献综述,目的是提高对AHA诊断和治疗的认识和知识。例演示。我们在这些病例报告中提出了三例诊断为AHA的患者。第一位患者为年轻女性,第二位和第三位患者分别为中老年男性。所有患者均以无关节积血和出血史的瘀伤为主诉。患者的实验室检查显示分离的aPTT延长,PT正常,存在抗因子VIII自身抗体,这些都是AHA的特征。患者随后接受皮质类固醇治疗以降低自身抗体滴度,并接受因子VIII输血以止血。结论:出现瘀伤症状而无关节积血和无出血史的患者可怀疑AHA。单独aPTT升高而PT正常应引起对AHA的高度怀疑。FVIII抑制剂的存在有助于确认AHA的诊断。治疗包括因子VIII输注和皮质类固醇治疗。建议使用旁路剂替代FVIII输液。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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Diagnosis and Management of Acquired Hemophilia A: Case Reports and a Literature Review.

Background: Acquired hemophilia A (AHA) is a potentially life-threatening autoimmune hemostatic disorder where autoantibodies that disrupt the functions of factor VIII (FVIII) are present in the circulation. The early diagnosis of AHA is difficult since the symptoms of AHA differ from those of congenital hemophilia A. Furthermore, the management of AHA is also more complex due to the presence of autoantibodies against FVIII (FVIII inhibitors). Here, we present three case reports and conduct a literature review of AHA with the aim to increase awareness and knowledge regarding the diagnosis and treatment of AHA. Case Presentations. We present three patients diagnosed with AHA in these case reports. The first patient was a young female, while the second and third patients were middle-aged and elderly males, respectively. All patients presented with a chief complaint of bruises without hemarthrosis and a history of bleeding. Laboratory examinations of the patients revealed isolated prolonged aPTT, normal PT, and the presence of autoantibodies against factor VIII, which are characteristics of AHA. Patients were then treated with corticosteroids to reduce the titer level of autoantibodies and received factor VIII transfusion to stop bleeding.

Conclusion: AHA can be suspected in patients presenting with symptoms of bruises without hemarthrosis and without the history of bleeding. Isolated aPTT elevation with normal PT should raise high suspicion of AHA. The presence of FVIII inhibitors can help to confirm the diagnosis of AHA. Treatment consists of factor VIII transfusion and corticosteroid therapy. Bypassing agents are recommended as an alternative to FVIII transfusion.

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来源期刊
Case Reports in Medicine
Case Reports in Medicine MEDICINE, GENERAL & INTERNAL-
CiteScore
1.70
自引率
0.00%
发文量
53
审稿时长
13 weeks
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