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获得性血友病 A 的独特出血模式和实验室发现的识别有助于及时治疗危及生命的疾病。

Recognition of the unique bleeding pattern and laboratory findings in acquired haemophilia A facilitates prompt treatment of a life-threatening disorder.

机构信息

School of Medicine, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.

Division of Hematology and Oncology, Medical College of Wisconsin, Milwaukee, Wisconsin, USA

出版信息

BMJ Case Rep. 2021 Aug 3;14(8):e244238. doi: 10.1136/bcr-2021-244238.

Abstract

Acquired haemophilia A (AHA) is an uncommon but severe acquired bleeding disorder caused by the development of antibodies against clotting factor VIII, impairing secondary haemostasis. It is more common in older individuals and characteristically presents with spontaneous soft tissue bleeding that can rapidly become life-threatening. Definitive treatment requires immunosuppression to eradicate anti-FVIII antibodies, while providing haemostatic support to manage bleeding. Transfusions of fresh frozen plasma or cryoprecipitate, typically used to treat severe bleeding, are ineffective in patients with AHA. Instead, highly specialised clotting factor concentrates are required. While the appearance and extent of the soft tissue bleeding and the markedly prolonged activated partial thromboplastin time are characteristic, lack of familiarity with this disease process can lead to significant treatment delays. We report the clinical course and management of a 65-year-old woman who presented with severe anaemia of unclear aetiology with unrecognised soft tissue bleeding who was subsequently diagnosed with AHA.

摘要

获得性血友病 A(AHA)是一种罕见但严重的获得性出血性疾病,由针对凝血因子 VIII 的抗体的发展引起,损害了二级止血。它在老年人中更为常见,其特征是自发性软组织出血,可迅速危及生命。明确的治疗需要免疫抑制以消除抗 FVIII 抗体,同时提供止血支持以控制出血。新鲜冷冻血浆或冷沉淀的输注通常用于治疗严重出血,但在 AHA 患者中无效。相反,需要高度专业化的凝血因子浓缩物。虽然软组织出血的外观和程度以及明显延长的激活部分凝血活酶时间是特征性的,但对这种疾病过程缺乏熟悉可能导致治疗明显延迟。我们报告了一位 65 岁女性的临床病程和治疗,她因不明病因的严重贫血就诊,伴有未被识别的软组织出血,随后被诊断为 AHA。

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