Hayashi Tomoe, Morishita Eriko, Asakura Hidesaku, Nakao Shinji
Department of Hematology, Kanazawa University Hospital, Japan.
Nihon Ronen Igakkai Zasshi. 2010;47(4):329-33. doi: 10.3143/geriatrics.47.329.
Acquired hemophilia A is a rare bleeding diathesis caused by autoantibodies against clotting factor VIII. The incidence of acquired hemophilia A increases with age. We report two cases of acquired hemophilia A in elderly patients and their clinical characteristics. Case 1: A 66-year-old man was referred to our hospital with massive subcutaneous and intramuscular hemorrhage. Prolonged APTT, low factor VIII activity and factor VIII inhibitor with high titer (42 BU/ml ) were observed, confirming the diagnosis of acquired hemophilia A. His hemorrhages disappeared soon after 50 mg/day oral prednisolone was administered. Although early steroid withdrawal lead to repeated prolongation of APTT, the addition of 20 mg/day oral prednisolone successfully decreased the inhibitor titer. The underlying disease was not identified. Case 2: An 85-year-old man with advanced gastric cancer was referred to our division because of severe bleeding. His factor VIII inhibitor titer was 64 BU/ml . Activated prothrombin complex concentrates were used to control the bleeding. Initially, he did not seem to respond to 20 mg/day oral prednisolone, but a further 12 weeks of 20 mg/day prednisolone finally achieved normalization of his hemostatic parameters. Subsequently, he successfully underwent surgery for cancer. The responses to immunosuppressive therapy were very different in the two cases, probably because of the difference in the underlying diseases. The immunosuppressive therapy of acquired hemophilia A should be strictly tailored to the patient's characteristics to minimize treatment-related adverse effects.
获得性血友病A是一种由抗凝血因子VIII自身抗体引起的罕见出血性素质。获得性血友病A的发病率随年龄增长而增加。我们报告两例老年患者的获得性血友病A及其临床特征。病例1：一名66岁男性因大量皮下和肌肉内出血被转诊至我院。观察到活化部分凝血活酶时间（APTT）延长、因子VIII活性降低以及高滴度（42 BU/ml）的因子VIII抑制剂，确诊为获得性血友病A。口服泼尼松龙50 mg/天后，他的出血症状很快消失。尽管早期停用类固醇导致APTT反复延长，但加用口服泼尼松龙20 mg/天后成功降低了抑制剂滴度。潜在疾病未明确。病例2：一名患有晚期胃癌的85岁男性因严重出血被转诊至我科。他的因子VIII抑制剂滴度为64 BU/ml。使用活化凝血酶原复合物浓缩物控制出血。起初，他似乎对口服泼尼松龙20 mg/天无反应，但进一步12周每天20 mg泼尼松龙最终使他的止血参数恢复正常。随后，他成功接受了癌症手术。两例患者对免疫抑制治疗的反应差异很大，可能是由于潜在疾病不同。获得性血友病A的免疫抑制治疗应严格根据患者特点进行调整，以尽量减少治疗相关的不良反应。
