Ayoola Ayodele, Mohsini Wajahath, Aung Sein
Department of Medicine, Franklin Square Hospital Center, Baltimore, MD 21237, USA.
Acta Haematol. 2008;119(1):8-11. doi: 10.1159/000112838. Epub 2008 Jan 4.
Acquired factor VIII inhibitor is an antibody formed against FVIII and seen in patients without FVIII replacement therapy. This entity has been associated with both autoimmune disease and infections.
We report the case of a 65-year-old man with dermatomyositis who presented with a bleeding episode following venipuncture and hematuria. His coagulation parameters were followed as well as the inhibitor level. His acquired FVIII inhibitor was managed successfully with rituximab.
Our patient's bleeding episodes resolved and the inhibitor level was undetectable after 12 months. This is the first case of acquired FVIII inhibitor in association with dermatomyositis.
获得性凝血因子VIII抑制物是一种针对FVIII形成的抗体,见于未接受FVIII替代治疗的患者。该疾病与自身免疫性疾病和感染均有关联。
我们报告了一名65岁皮肌炎男性患者的病例,该患者在静脉穿刺后出现出血事件及血尿。我们对其凝血参数以及抑制物水平进行了监测。他的获得性FVIII抑制物通过利妥昔单抗成功得到控制。
我们患者的出血事件得到缓解,12个月后抑制物水平检测不到。这是首例与皮肌炎相关的获得性FVIII抑制物病例。