Shimizu Hiroaki, Saitoh Takayuki, Osaki Yohei, Yamane Arito, Gohda Fumito, Irisawa Hiroyuki, Yokohama Akihiko, Uchiumi Hideki, Handa Hiroshi, Matsushima Takafumi, Tsukamoto Norifumi, Karasawa Masamitsu, Murakami Hirokazu, Nojima Yoshihisa
Department of Medicine and Clinical Science, Gunma University, Graduate School of Medicine.
Rinsho Ketsueki. 2007 Jun;48(6):514-7.
We present a case of a 74-year-old male, who had a relapse of minimal change nephrotic syndrome (MCNS) as the initial presentation of acquired hemophilia A. MCNS had been maintained in remission with prednisolone 10 mg for 15 years. In early December 2005, the patient developed edema of the right leg, was admitted to a local general hospital, and was diagnosed as having a relapse of MCNS based on massive proteinuria (urine protein 6.1 g/day). One week later, severe anemia (hemoglobin 4.4 g/dl) and acute renal failure (creatinine 2.0 mg/dl) developed, and a CT scan of the abdomen revealed a hematoma in the left iliopsoas muscle. He was referred to our hospital with bleeding tendency. Laboratory examination revealed prolonged APTT 80.5 seconds), reduced factor VIII activity (<1%) and thepresence of factor VIII inhibitor at a titer of 19 Bethesda units/ml, based on which he was diagnosed as having acquired hemophilia A. With recombinant activated FVII, hemostasis was obtained and prednisolone administration 60 mg/day (1 mg/kg) was started. Both the acquired hemophilia A and MCNS responded well to the treatment with prednisolone. Six weeks after initiation of the treatment, factor VIII inhibitor and urine protein disappeared. This patient is considered to be a rare case; to the best of our knowledge, this is the third report of acquired hemophilia A with nephrotic syndrome.
我们报告一例74岁男性患者，其以获得性血友病A的首发表现为微小病变肾病综合征（MCNS）复发。MCNS曾用10mg泼尼松龙维持缓解15年。2005年12月初，患者出现右腿水肿，入住当地综合医院，基于大量蛋白尿（尿蛋白6.1g/天）被诊断为MCNS复发。一周后，出现严重贫血（血红蛋白4.4g/dl）和急性肾衰竭（肌酐2.0mg/dl），腹部CT扫描显示左髂腰肌血肿。他因出血倾向被转诊至我院。实验室检查显示活化部分凝血活酶时间延长（80.5秒），因子VIII活性降低（<1%），且存在滴度为19 Bethesda单位/ml的因子VIII抑制剂，据此诊断为获得性血友病A。使用重组活化FVII后实现止血，并开始给予泼尼松龙60mg/天（1mg/kg）。获得性血友病A和MCNS对泼尼松龙治疗反应良好。治疗开始六周后，因子VIII抑制剂和尿蛋白消失。该患者被认为是罕见病例；据我们所知，这是第三例关于获得性血友病A合并肾病综合征的报告。
