Rech Jürgen, Hueber Axel J, Leipe Jan, Manger Bernhard, Schett Georg, Kallert Stefan
Medical Department 3, Division of Rheumatology, University of Erlangen-Nuremberg, Munich, Germany.
Ther Apher Dial. 2008 Oct;12(5):406-8. doi: 10.1111/j.1744-9987.2008.00618.x.
Induction of factor VIII (FVIII) inhibitors sometimes occurs in patients with hemophilia due to frequent supplementation of FVIII. The inhibitor is rarely detected in non-hemophilic patients; however, an association has been described in patients with chronic inflammatory diseases, such as autoimmune diseases (e.g. SLE and rheumatoid arthritis), malignant tumors and drug allergies, and also to pregnant or aged individuals without underlying disease. We report on an 82-year-old patient who was transferred to our hospital after the diagnosis of acquired FVIII inhibitor. On admission the laboratory results showed no detectable FVIII activity (0%, normal range 70-100%), prolongation of coagulation time (APTT 102.4 s), and severe anemia 7.8 g/dL (normal range 12-16 g/dL). On physical examination multiple subcutaneous hematomas were detected and further bleeding in his left pectoralis muscle was observed. Despite extensive investigation no underlying disease was detected. Thirty-six courses of plasma exchange with 360 units of fresh frozen plasma replacement daily were conducted. High dose steroids and mycophenolate mofetil (MMF) were given throughout the course, and cyclophosphamide was administered once. Thirty-four units of erythrocytes were applied during this time. After 36 courses of plasma exchange in combination with high dose steroids, FVIII activity and coagulation time normalized and bleeding could be stopped. The patient was discharged in good health 48 days after admission. Hence, we present a case of severe idiopathic FVIII inhibitor-positive hemophilia successfully treated with the combination of plasma exchange, corticosteroids, cyclophosphamide and MMF.
由于频繁补充凝血因子 VIII（FVIII），血友病患者有时会出现 FVIII 抑制剂。在非血友病患者中很少检测到这种抑制剂；然而，在患有慢性炎症性疾病的患者中，如自身免疫性疾病（如系统性红斑狼疮和类风湿性关节炎）、恶性肿瘤和药物过敏患者，以及无基础疾病的孕妇或老年人中，也有相关报道。我们报告一例 82 岁患者，在诊断为获得性 FVIII 抑制剂后转至我院。入院时实验室结果显示未检测到 FVIII 活性（0%，正常范围 70 - 100%），凝血时间延长（活化部分凝血活酶时间 102.4 秒），以及严重贫血 7.8 g/dL（正常范围 12 - 16 g/dL）。体格检查发现多处皮下血肿，并观察到其左胸大肌有进一步出血。尽管进行了广泛检查，但未发现基础疾病。进行了 36 个疗程的血浆置换，每天用 360 单位新鲜冰冻血浆替代。在整个疗程中给予高剂量类固醇和霉酚酸酯（MMF），并给予一次环磷酰胺。在此期间输注了 34 单位红细胞。经过 36 个疗程的血浆置换联合高剂量类固醇治疗后，FVIII 活性和凝血时间恢复正常，出血得以停止。患者入院 48 天后健康出院。因此，我们报告了一例严重特发性 FVIII 抑制剂阳性血友病患者，通过血浆置换、皮质类固醇、环磷酰胺和 MMF 联合治疗成功治愈。
