Guerrero Camacho Raisa, Álvarez Román María Teresa, Butta Coll Nora, Zagrean Damaris, Rivas Pollmar Isabel, Martín Salces Mónica, Gasior Kabat Mercedes, Jiménez-Yuste Víctor
Servicio de Hematología y Hemoterapia, Hospital Universitario La Paz, Paseo de la Castellana 231, 28046 Madrid, Spain.
Coagulopathies and Haemostasis Disorders Group, IdiPAZ, Paseo de la Castellana 231, 28046 Madrid, Spain.
J Clin Med. 2022 May 11;11(10):2721. doi: 10.3390/jcm11102721.
Acquired haemophilia A (AHA) is a rare severe bleeding disorder resulting from the production of autoantibodies directed against coagulation factor VIII. At presentation, bleeding events can be severe, and an early diagnosis and treatment are of major importance. The current study aims to analyse the treated patients who have been diagnosed with AHA for a better understanding of our population and treatment outcome. We conducted a retrospective study with 26 patients who had been diagnosed with AHA and who were treated in our hospital between January 2006 and January 2021. The patients ranged in age from 30 to 85 years old: 46.10% were men, 46.10% had no known underlying condition, 27% had an underlying malignancy, 7.60% presented with other diseases: psoriatic arthritis and Paget's disease, and 19.30% presented with AHA during puerperium. All of the patients had bleeding events and were treated with bypass agents for this as well as with immunosuppressive therapy to eradicate the inhibitor. A total of 53.80% of the patients had major bleeding. Sixty-nine percent of the patients achieved complete remission, but 26.90% died during the follow-up, although bleeding was not the cause of death in any of these cases. Our observations underline the importance of clinical suspicion and early referral to centres with experience and laboratory facilities for managing AHA.
获得性血友病A（AHA）是一种罕见的严重出血性疾病，由针对凝血因子VIII的自身抗体产生所致。就诊时，出血事件可能很严重，早期诊断和治疗至关重要。本研究旨在分析已确诊为AHA的接受治疗的患者，以便更好地了解我们的患者群体和治疗结果。我们对2006年1月至2021年1月期间在我院确诊并接受治疗的26例AHA患者进行了回顾性研究。患者年龄在30至85岁之间：46.10%为男性，46.10%无已知基础疾病，27%有潜在恶性肿瘤，7.60%患有其他疾病：银屑病关节炎和佩吉特病，19.30%在产褥期出现AHA。所有患者均有出血事件，并为此接受了旁路制剂治疗以及免疫抑制治疗以消除抑制物。共有53.80%的患者发生大出血。69%的患者实现完全缓解，但26.90%的患者在随访期间死亡，尽管在这些病例中出血均不是死亡原因。我们的观察结果强调了临床怀疑以及早期转诊至有经验和实验室设施的中心来管理AHA的重要性。
