Goto Sawako, Ookawara Susumu, Ueda Yuichiro, Ito Kiyonori, Miyazawa Haruhisa, Kitano Taisuke, Shindo Mitsutoshi, Kaku Yoshio, Hirai Keiji, Hoshino Taro, Ashizawa Masahiro, Mori Honami, Yoshida Izumi, Tabei Kaoru
Division of Nephrology, First Department of Integrated Medicine, Saitama Medical Center, Jichi Medical University, 1-847 Amanuma-cho, Omiya-ku, Saitama-City, Saitama, 330-8503, Japan.
Division of Hematology, First Department of Integrated Medicine, Saitama Medical Center, Jichi Medical University, Saitama, Japan.
CEN Case Rep. 2015 Nov;4(2):196-199. doi: 10.1007/s13730-015-0168-z. Epub 2015 Jan 25.
An 80-year-old man was treated at our hospital for chronic kidney disease, diabetes mellitus and hypertension. He presented a bleeding tendency over the past several weeks. When he was admitted to our hospital with a consciousness disturbance, he had irregular 10-20 cm patches of subcutaneous bleeding. Laboratory findings showed prolonged activated partial thromboplastin time (APTT) but normal platelet count and prothrombin time. The administration of fresh frozen plasma did not control the bleeding tendency and the result of APTT cross-mixing test suggested the presence of factor VIII inhibitor. The diagnosis of acquired hemophilia A was made by a marked decrease in factor VIII activity levels (<1 %) and a high titer of factor VIII inhibitor (11 Bethesda units). To remove the factor VIII inhibitor and suppress its production, plasma exchange was performed and methylprednisolone was administered. Thereafter, the bleeding tendency could be controlled with APTT measurement normalization. Plasma exchange does not have a first priority in the treatment but our case findings suggested that plasma exchange is an effective method for eliminating factor VIII inhibitor and alleviating acquired hemophilia A severity.
一名80岁男性因慢性肾脏病、糖尿病和高血压在我院接受治疗。在过去几周里，他出现了出血倾向。当他因意识障碍入院时，有不规则的10 - 20厘米皮下出血斑。实验室检查结果显示活化部分凝血活酶时间（APTT）延长，但血小板计数和凝血酶原时间正常。输注新鲜冰冻血浆未能控制出血倾向，APTT交叉配血试验结果提示存在Ⅷ因子抑制剂。通过Ⅷ因子活性水平显著降低（<1%）和高滴度的Ⅷ因子抑制剂（11贝塞斯达单位）确诊为获得性血友病A。为了去除Ⅷ因子抑制剂并抑制其产生，进行了血浆置换并给予甲泼尼龙。此后，随着APTT测量恢复正常，出血倾向得到了控制。血浆置换在治疗中并非首要选择，但我们的病例结果表明，血浆置换是消除Ⅷ因子抑制剂和减轻获得性血友病A严重程度的有效方法。
