Department of Transfusion Medicine, General County Hospital Vinkovci, Vinkovci, Croatia.
Faculty of Medicine, University of Osijek, Osijek, Croatia.
Biochem Med (Zagreb). 2022 Oct 1;32(3):030801. doi: 10.11613/BM.2022.030801. Epub 2022 Aug 5.
The acquired hemophilia A (AHA) is a life-threatening condition. The incidence of AHA is extremely low, which requires a multidisciplinary approach to diagnosis and treatment. This is case report of 73-year-old man who presented with AHA secondary to severe acute respiratory syndrome Coronavirus 2 (SARS-CoV-2) pneumonia. The patient had extensive skin bleeding and hematomas. In the coagulation screening tests activated partial thromboplastin time (APTT) was prolonged with normal prothrombin time (PT), which was indication for further investigation. The APTT in a mixing study with normal plasma did not correct so clotting factors inhibitors were suspected. With signs of bleeding, extremely low factor VIII (FVIII) activity (2%) and presence of FVIII inhibitors, AHA was diagnosed and treatment initiated. Patient was treated with factor eight inhibitor bypassing agent (FEIBA) for three days, followed by long-term corticosteroid and cyclophosphamide therapy. Malignant and autoimmune diseases as the most common causes of AHA were ruled out. The patient had a good response to therapy with gradual normalization of APTT and FVIII activity. To the best of our knowledge, the present case is the first reported case of AHA after SARS-CoV-2 pneumonia. The diagnosis of AHA should be suspected in a patient with bleeding into the skin and mucous membranes without a previous personal and family history of bleeding, and with isolated prolonged APTT. It is important to investigate any isolated prolongation of APTT in cooperation with clinical laboratory experts.
获得性血友病 A(AHA)是一种危及生命的疾病。AHA 的发病率极低,这需要多学科方法进行诊断和治疗。这是一例 73 岁男性患者,因严重急性呼吸综合征冠状病毒 2(SARS-CoV-2)肺炎继发 AHA 的病例报告。患者有广泛的皮肤出血和血肿。在凝血筛选试验中,活化部分凝血活酶时间(APTT)延长,而凝血酶原时间(PT)正常,这表明需要进一步检查。在与正常血浆的混合研究中,APTT 未得到纠正,因此怀疑存在凝血因子抑制剂。由于有出血迹象、极低的因子 VIII(FVIII)活性(2%)和 FVIII 抑制剂的存在,诊断为 AHA 并开始治疗。患者接受了因子八抑制剂旁路剂(FEIBA)治疗 3 天,随后进行长期皮质类固醇和环磷酰胺治疗。恶性和自身免疫性疾病是 AHA 的最常见原因,已被排除。患者对治疗反应良好,APTT 和 FVIII 活性逐渐恢复正常。据我们所知,目前的病例是 SARS-CoV-2 肺炎后首例 AHA 报告。在没有先前个人和家族出血史的情况下,当患者出现皮肤和黏膜出血且 APTT 孤立延长时,应怀疑 AHA 的诊断。与临床实验室专家合作,对任何孤立的 APTT 延长进行调查非常重要。