Department of Internal Medicine, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan.
Education Center, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan.
J Formos Med Assoc. 2022 Sep;121(9):1872-1876. doi: 10.1016/j.jfma.2022.02.017. Epub 2022 Mar 14.
Acquired hemophilia is a rare disease resulting from autoantibodies against endogenous factor VIII (FVIII), which associates with bleeding and a high mortality rate. The pathophysiology is still unclear. Recent studies suggest genetic and environmental factors trigger the breakdown of immune tolerance. We report a 77-year-old Taiwanese man presented with multiple ecchymoses and some hemorrhagic blisters three weeks after SARS-CoV-2 mRNA (Moderna) vaccination. Isolated activated partial thromboplastin time (aPTT) prolongation was found. Acquired hemophilia A (AHA) was confirmed by low factor VIII (FVIII) activity and high titer of FVIII inhibitor. The pathohistology of skin biopsy further supported the concomitant diagnosis of bullous pemphigoid. To date, 6 cases of acquired hemophilia A following SARS-CoV-2 mRNA vaccination were reported worldwide. We reviewed and summarized the characteristics of these cases. We also discussed the rare finding of concomitant acquired hemophilia A and bullous pemphigoid. Bullous pemphigoid results from autoantibody against epithelial basement membrane zone of skin. In this article, we proposed possibility of SARS-CoV-2 mRNA vaccine associated autoimmunity against FVIII and epithelial basement membrane zone.
获得性血友病是一种罕见的疾病,由针对内源性因子 VIII (FVIII) 的自身抗体引起,与出血和高死亡率有关。其病理生理学仍不清楚。最近的研究表明,遗传和环境因素引发了免疫耐受的破坏。我们报告了一位 77 岁的台湾男性,在接种 SARS-CoV-2 mRNA(Moderna)疫苗后三周出现多处瘀斑和一些出血性水疱。发现孤立的活化部分凝血活酶时间 (aPTT) 延长。通过低因子 VIII (FVIII) 活性和高 FVIII 抑制剂滴度确诊获得性血友病 A (AHA)。皮肤活检的病理组织学进一步支持大疱性类天疱疮的并发诊断。迄今为止,全世界已报告了 6 例接种 SARS-CoV-2 mRNA 疫苗后发生的获得性血友病 A 病例。我们回顾并总结了这些病例的特征。我们还讨论了罕见的同时发生获得性血友病 A 和大疱性类天疱疮的情况。大疱性类天疱疮是由针对皮肤上皮基底膜带的自身抗体引起的。在本文中,我们提出了 SARS-CoV-2 mRNA 疫苗可能与 FVIII 和上皮基底膜带的自身免疫有关的可能性。