产后获得性血友病A:约3例

Widad Rhandour, Hajar Masrour, Latifa Laaghrib, N. Oubelkacem, Z. Khammar, R. Berrady
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引用次数: 0

摘要

获得性血友病A (AHA)是一种罕见但严重的自身免疫性疾病。它是由获得因子VIII (FVIII)自身抗体引起的。这些产后自身抗体占所有AHA病例的7-21%,最常在第一次怀孕后发生。我们报告了3例产后获得性血友病a的新病例。观察:3例无病理史的年轻女性表现为自发性和异质性出血综合征,2例深血肿,第三例患者血尿伴瘀斑。所有患者的诊断均基于血浆FVIII活性<50%和FVIII抑制剂的存在。所有患者免疫检查和肿瘤研究均为阴性。3例患者均采用止血联合糖皮质激素治疗及免疫抑制药物治疗。结论:AHA是一种罕见的疾病,其诊断在我国人群中经常被低估。这是至关重要的,使临床医生意识到这种病理,并能够唤起它在育龄的年轻患者。在以后的怀孕中有可能复发,因此建议长期随访。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Acquired Postpartum Hemophilia A: About 3 Cases
Introduction: Acquired hemophilia A (AHA) is a rare but severe autoimmune disorder. It is caused by the acquisition of autoantibodies to factor VIII (FVIII). These post-partum autoantibodies account for 7-21% of all cases of AHA and most often develop after the first pregnancy. We report 3 new cases of postpartum-acquired hemophilia A. Observations: Three young women with no prior pathological history presented with a spontaneous and heterogeneous hemorrhagic syndrome, two cases of deep hematomas, and haematuria with ecchymosis in the 3rd patient. The diagnosis was made in all our patients based on plasma FVIII activity <50% and the presence of an FVIII inhibitor. Immunological tests and neoplastic research were negative in all our patients. Hemostatic treatment combined with corticosteroid therapy and immunosuppressive drugs were used for the 3 patients. Conclusion: AHA is a rare disease, and its diagnosis is often underestimated in our population. It is essential to make clinicians aware of this pathology and to be able to evoke it in young patients of childbearing age. A relapse is possible in future pregnancies, so long-term follow-up is recommended.
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