Taza Fadi, Suleman Nawar, Paz Robert, Haas Christopher
Department of Medicine, MedStar Health Internal Medicine Residency Program, Baltimore, MD, USA.
Department of Medicine, MedStar Health Hospitalist Program, Baltimore, MD, USA.
J Community Hosp Intern Med Perspect. 2021 Jan 26;11(1):89-93. doi: 10.1080/20009666.2020.1836726.
Acquired Hemophilia A (AHA) is a rare entity, resulting from the production of autoantibodies against Factor VIII of the coagulation cascade. These autoantibodies may develop in response to autoimmune conditions, drugs, neoplastic diseases, and pregnancy. Diagnosis involves clinical presentation, mucocutaneous or intramuscular bleeding, and laboratory findings, such as prolonged activated partial thromboplastin time, decreased levels of Factor VIII, and the presence of Factor VIII autoantibodies. The etiology is diverse, with a variety of underlying culprits. Malignancy-associated AHA has been associated with approximately 15% of cases. Urothelial malignancy-mediated AHA is exceedingly rare, with only two previously published reports. The management of AHA includes stabilization and control of bleeding via the use of hemostatic agents, and elimination of the inhibitor with immunosuppressive therapy. Here, we report a case of AHA secondary to urothelial malignancy and review the pathobiology and pathogenesis of Hemophilia A and AHA.
获得性血友病A(AHA)是一种罕见疾病,由针对凝血级联反应中凝血因子VIII产生自身抗体所致。这些自身抗体可能因自身免疫性疾病、药物、肿瘤性疾病及妊娠而产生。诊断包括临床表现、黏膜皮肤或肌肉内出血以及实验室检查结果,如活化部分凝血活酶时间延长、凝血因子VIII水平降低以及存在凝血因子VIII自身抗体。病因多种多样,有多种潜在因素。与恶性肿瘤相关的AHA约占病例的15%。尿路上皮恶性肿瘤介导的AHA极为罕见,此前仅有两篇报道。AHA的治疗包括使用止血剂稳定和控制出血,以及通过免疫抑制疗法消除抑制剂。在此,我们报告一例继发于尿路上皮恶性肿瘤的AHA病例,并综述血友病A和AHA的病理生物学及发病机制。