Ianuà Eleonora, Caldarelli Mario, De Matteis Giuseppe, Cianci Rossella, Gambassi Giovanni
Department of Translational Medicine and Surgery, Catholic University, Fondazione Policlinico Universitario "Agostino Gemelli", IRCCS, 00168 Rome, Italy.
Diseases. 2023 Dec 24;12(1):5. doi: 10.3390/diseases12010005.
Hemophilia A is a hemorrhagic disorder caused by insufficient or inadequate coagulation factor VIII activity. Two different forms are described: congenital, hereditary X-linked, and acquired. Acquired hemophilia A (AHA) is a rare condition and it is defined by the production of autoantibodies neutralizing factor VIII, known as inhibitors. We report the case of a 72-year-old man with a clinical diagnosis of AHA after SARS-CoV-2 infection, which has been described in association with several hematological complications. SARS-CoV-2 infection could represent the immunological trigger for the development of autoantibodies. In our patient, SARS-CoV-2 infection preceded the hemorrhagic complications by 15 days. This lag time is in line with the other cases reported and compatible with the development of an intense immune response with autoantibody production. It is possible that since our patient was affected by type 1 diabetes mellitus, he was more prone to an immune system pathological response against self-antigens. A prompt, appropriate therapeutic intervention with activated recombinant factor VII administration and cyclophosphamide has led to rapid remission of clinical and laboratory findings.
甲型血友病是一种由于凝血因子 VIII 活性不足或缺乏引起的出血性疾病。它有两种不同形式：先天性、遗传性 X 连锁型和获得性。获得性甲型血友病（AHA）是一种罕见疾病，其定义为产生中和因子 VIII 的自身抗体，即抑制剂。我们报告了一例 72 岁男性患者，在感染 SARS-CoV-2 后临床诊断为 AHA，此前已有 SARS-CoV-2 感染与多种血液学并发症相关的报道。SARS-CoV-2 感染可能是自身抗体产生的免疫触发因素。在我们的患者中，SARS-CoV-2 感染比出血并发症早 15 天。这段间隔时间与其他报道的病例一致，并且与产生自身抗体的强烈免疫反应的发展相符。由于我们的患者患有 1 型糖尿病，他可能更容易出现针对自身抗原的免疫系统病理反应。通过给予活化重组因子 VII 和环磷酰胺进行迅速、适当的治疗干预，已使临床和实验室检查结果迅速缓解。
