Dürig J, de Wit M, Fiedler W, Marx G, Hossfeld D K
Abteilung Onkologie und Hämatologie, Medizinische Klinik und Poliklinik, Hamburg.
Schweiz Med Wochenschr. 1996 Nov 23;126(47):2026-31.
A 59-year-old patient who presented with hematuria and recurrent soft tissue bleeding was found to have a factor VIII inhibitor level of 52 Bethesda units (BU)/ml and acquired hemophilia was diagnosed. After treatment with immunoglobulins (0.4 g IgG/kg per day for one week) the factor VIII inhibitor titer decreased to 12 BU/ml. Because of another episode of retroperitoneal hemorrhage, the patient was put on an immunosuppressive combination therapy which was first described by Lian et al. (1988). Our patient was infused with a factor VIII concentrate followed by cyclophosphamide, vincristine and prednisone. This regimen was repeated every 3-4 weeks. After 6 courses a further decline in the factor VIII inhibitor concentration, but no complete eradication of the autoantibody, was achieved. The factor VIII inhibitor level has remained at 2.5 BU/ml for more than 7 weeks without further bleeding episodes. The pathophysiology and treatment of acquired hemophilia are discussed.
一名59岁出现血尿和反复软组织出血的患者，其VIII因子抑制物水平为52贝塞斯达单位（BU）/毫升，被诊断为获得性血友病。在接受免疫球蛋白治疗（每天0.4克IgG/千克，持续一周）后，VIII因子抑制物滴度降至12 BU/毫升。由于再次发生腹膜后出血，该患者接受了Lian等人（1988年）首次描述的免疫抑制联合治疗。我们的患者先输注VIII因子浓缩物，随后给予环磷酰胺、长春新碱和泼尼松。该方案每3 - 4周重复一次。6个疗程后，VIII因子抑制物浓度进一步下降，但自身抗体未完全清除。VIII因子抑制物水平在超过7周的时间里一直维持在2.5 BU/毫升，未再出现出血发作。文中讨论了获得性血友病的病理生理学和治疗方法。
