Khoulani Dania, Rao Bharat, Khanshour Ammar, Kuriakose Philip, Yessayan Lenar
Department of Internal Medicine, Henry Ford Hospital, 2799 W. Grand Boulevard, Detroit, MI 48202, USA.
Department of Internal Medicine, Division of Hospital Medicine, Henry Ford Hospital, 2799 W. Grand Boulevard, Detroit, MI 48202, USA.
Case Rep Hematol. 2014;2014:283086. doi: 10.1155/2014/283086. Epub 2014 Jul 10.
Diffuse alveolar hemorrhage (DAH) is a serious complication of the small vessel vasculitis syndromes and carries a high mortality. Recombinant activated factor VII (rFVIIa) is used to treat bleeding in patients with hemophilia and antibodies to factor VIII or IX. It is increasingly being used in life-threatening hemorrhage in a variety of other settings in which conventional therapy is unsuccessful. Randomized controlled trials of rFVIIa in DAH are lacking. However, several case reports have described a complete or sustained control of DAH using rFVIIa after patients failed to respond to medical treatment. There are no case reports in the literature describing the use or the failure of rFVIIa in DAH associated with cryoglobulinemic vasculitis. We here report the failure of rFVIIa to control DAH in a patient with CD5+ B-cell non-Hodgkin's lymphoma and cryoglobulinemic vasculitis.
弥漫性肺泡出血(DAH)是小血管血管炎综合征的一种严重并发症,死亡率很高。重组活化因子VII(rFVIIa)用于治疗血友病患者以及存在抗VIII或IX因子抗体患者的出血。在常规治疗失败的各种其他危及生命的出血情况中,它的使用越来越多。目前缺乏关于rFVIIa治疗DAH的随机对照试验。然而,一些病例报告描述了在患者对药物治疗无反应后,使用rFVIIa可使DAH得到完全或持续控制。文献中没有关于rFVIIa在冷球蛋白血症性血管炎相关DAH中的使用或失败的病例报告。我们在此报告1例CD5 + B细胞非霍奇金淋巴瘤合并冷球蛋白血症性血管炎患者,rFVIIa未能控制DAH。