Zdziarska Joanna, Musiał Jacek
Pol Arch Med Wewn. 2014;124(4):200-6. doi: 10.20452/pamw.2192. Epub 2014 Mar 21.
Acquired hemophilia is a rare bleeding disorder caused by autoantibodies that inhibit coagulation factor VIII. In most cases, it manifests with severe, often life‑threatening bleeds. Acquired hemophilia may be idiopathic or secondary to another condition, most commonly other autoimmune disease or cancer. Treatment is directed to stop bleeding and eradicate inhibitory autoantibodies. Like in most life‑threatening conditions, early diagnosis and treatment are essential for good prognosis. Prompt diagnosis and treatment of acquired hemophilia are constantly improving owing to the increasing availability of laboratory diagnostic tests and growing awareness of physicians of various specialties.
获得性血友病是一种由抑制凝血因子VIII的自身抗体引起的罕见出血性疾病。在大多数情况下,它表现为严重的、常常危及生命的出血。获得性血友病可能是特发性的,也可能继发于另一种疾病,最常见的是其他自身免疫性疾病或癌症。治疗旨在止血并根除抑制性自身抗体。与大多数危及生命的疾病一样,早期诊断和治疗对于良好的预后至关重要。由于实验室诊断测试的可及性不断提高以及各专业医生的认识不断增强,获得性血友病的及时诊断和治疗正在不断改善。