Department of Internal Medicine, Centro Hospitalar Universitário de São João, Alameda Prof. Hernâni Monteiro, 4200-319, Porto, Portugal.
Department of Internal Medicine, Centro Hospitalar Universitário de São João, Alameda Prof. Hernâni Monteiro, 4200-319, Porto, Portugal.
Transfus Apher Sci. 2022 Apr;61(2):103337. doi: 10.1016/j.transci.2021.103337. Epub 2021 Nov 30.
Acquired Hemophilia A (AHA) is a rare autoimmune disorder, caused by the development of circulating autoantibodies against coagulation factor VIII (FVIII). AHA is associated with bullous pemphigoid in 2% of patients. We report a case of a 74-year-old man admitted with anemia and a tense subcutaneous and muscular hematoma in the right thigh. Blood analysis confirmed AHA. The patient had a recent diagnosis of bullous pemphigoid. Response to bypass agents and corticosteroids was good with bleeding control and normalization of FVIII and negative autoantibodies, respectively. In a 3-month follow-up period after tapering and stopping prednisolone, a relapse occurred, and immunosuppression was reinitiated. An early diagnosis and effective treatment in AHA are essential to reduce morbimortality. A careful tapering of immunosuppression is important to minimize FVIII inhibitor recurrence, as observed in this case.
获得性血友病 A（AHA）是一种罕见的自身免疫性疾病，由凝血因子 VIII（FVIII）循环自身抗体的发展引起。AHA 与大疱性类天疱疮有关，占患者的 2%。我们报告了一例 74 岁男性患者，因贫血和右大腿紧张性皮下和肌肉血肿入院。血液分析证实为 AHA。患者最近被诊断为大疱性类天疱疮。旁路制剂和皮质类固醇的反应良好，分别控制出血并使 FVIII 和自身抗体正常化。在逐渐减少和停用泼尼松龙后的 3 个月随访期间，复发发生，并重新开始免疫抑制。早期诊断和有效治疗 AHA 对于降低发病率和死亡率至关重要。正如本例所示，仔细减少免疫抑制剂的用量对于最大限度地减少 FVIII 抑制剂的复发非常重要。
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