产后获得性血友病A并发深静脉血栓1例。

Q3 Medicine
Amitabha Saha, Md Abid Sarfaraz, Arjun Talapatra, Sushmita Basu, Nausheen Arshad
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引用次数: 0

摘要

获得性血友病A (AHA)是一种罕见的自身免疫性疾病(百万分之1.5),由抗因子VIII的自身抗体引起,导致凝血功能障碍。产后AHA并不常见,仅占1-5%的病例,典型表现为皮肤粘膜或阴道出血,而深静脉血栓形成(DVT)是一种极为罕见的表现。我们报告一例33岁的产后女性表现为左下肢深静脉血栓和自发性出血。最初使用肝素和直接口服抗凝剂(DOACs)加重了出血,促使修改治疗方案。在血小板计数和凝血酶原时间正常的情况下,持续升高的活化部分凝血酶时间(aPTT)增加了对AHA的怀疑。Bethesda试验证实高滴度因子VIII抑制剂(35.2 BU/mL)。利妥昔单抗、皮质类固醇和环磷酰胺的免疫抑制治疗成功地导致了缓解。本病例强调了一个不寻常的表现AHA与DVT在产后患者。血栓和出血的合并需要仔细诊断和个体化治疗。及时识别aPTT延长,未校正的混合研究和确认Bethesda试验是早期干预的关键。产后AHA,虽然罕见,但应考虑产后女性凝血功能障碍或血栓形成。早期诊断和量身定制的治疗可改善结果并降低死亡率。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A Rare Case of Postpartum-acquired Hemophilia A Presenting with Deep Vein Thrombosis.

Acquired hemophilia A (AHA) is a rare autoimmune disorder (1.5 per million) caused by autoantibodies against factor VIII, leading to coagulopathy. Postpartum AHA is uncommon, accounting for only 1-5% of cases, and typically presents with mucocutaneous or vaginal bleeding, while deep vein thrombosis (DVT) is an extremely rare manifestation. We report a case of a 33-year-old postpartum female presenting with left lower limb DVT and spontaneous bleeding. Initial management with heparin and direct oral anticoagulants (DOACs) exacerbated bleeding, prompting treatment revision. Persistently elevated activated partial thromboplastin time (aPTT) with normal platelet count and prothrombin time (PT) raised suspicion of AHA. A Bethesda assay confirmed high-titer factor VIII inhibitor (35.2 BU/mL). Immunosuppressive therapy with rituximab, corticosteroids, and cyclophosphamide successfully led to remission. This case highlights an unusual presentation of AHA with DVT in a postpartum patient. The combination of thrombosis and bleeding necessitates careful diagnosis and individualized management. Prompt recognition of prolonged aPTT, uncorrected mixing study, and confirmatory Bethesda assay are crucial for early intervention. Postpartum AHA, though rare, should be considered in postpartum females presenting with coagulopathy or thrombosis. Early diagnosis and tailored treatment improve outcomes and reduce mortality.

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