University of Arkansas for Medical Sciences, Little Rock, AR, USA.
Department of Internal Medicine, University of Arkansas for Medical Sciences, Little Rock, AR, USA.
Perm J. 2022 Jun 29;26(2):153-157. doi: 10.7812/TPP/21.109. Epub 2022 Jun 15.
Introduction Acquired hemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies against clotting factor VIII. It occurs most commonly in the elderly population. AHA is associated with malignancy, autoimmune diseases, pregnancy, and drugs. However, up to 50% of reported cases remain idiopathic. AHA is very rarely associated with multiple sclerosis. Case Presentation A 56-year-old patient with a history of MS presented with thigh and arm swelling and associated pain. Imaging revealed diffuse iliopsoas hematoma. Laboratory studies showed that prothombin time was not elevated and there was prolongation of activated partial thromboplastin time that was not corrected by mixing studies. Clotting factor VIII activity level was reduced, and the inhibitor level was elevated. He was treated with factor eight inhibitor bypassing agents, rituximab, and steroids. Conclusion AHA should be suspected in any patient with new-onset bleeding in the presence of a prolonged activated partial thromboplastin time. Prompt diagnosis and treatment are important as AHA carries a high mortality rate.
简介
获得性血友病 A(AHA)是一种由针对凝血因子 VIII 的自身抗体引起的罕见出血性疾病。它最常见于老年人群。AHA 与恶性肿瘤、自身免疫性疾病、妊娠和药物有关。然而,高达 50%的报告病例仍然是特发性的。AHA 非常罕见与多发性硬化症相关。
病例介绍
一位 56 岁有 MS 病史的患者出现大腿和手臂肿胀和相关疼痛。影像学显示弥漫性髂腰肌血肿。实验室研究表明凝血酶原时间不升高,且激活部分凝血活酶时间延长,混合研究无法纠正。凝血因子 VIII 活性水平降低,抑制剂水平升高。他接受了因子八抑制剂旁路剂、利妥昔单抗和类固醇治疗。
结论
在存在延长的激活部分凝血活酶时间的情况下,任何新出现出血的患者都应怀疑 AHA。及时诊断和治疗很重要,因为 AHA 死亡率很高。