Ichikawa Satoshi, Saito Kei, Fukuhara Noriko, Tanaka Yuya, Lee Yoonha, Onodera Koichi, Onishi Yasushi, Yokoyama Hisayuki, Fujiwara Minami, Harigae Hideo
Department of Hematology and Rheumatology, Tohoku University Hospital, Japan.
Intern Med. 2020 May 15;59(10):1303-1308. doi: 10.2169/internalmedicine.4142-19. Epub 2020 Feb 5.
Acquired factor X deficiency (AFXD) is a very rare coagulation disorder. A 40-year-old man with no comorbidities suffering from a fever, malaise, and severe hemorrhagic symptoms, including massive hematuria, was emergently admitted. His platelet count was normal, but his prothrombin time and activated partial thromboplastin time were markedly prolonged, which was thought to be due to autoantibody against a coagulation factor in the common pathway. Despite severe massive hematuria resulting in transient renal failure, he was successfully treated with urgent immunosuppressive therapy. Computed tomography revealed bronchopneumonia, which improved with antibiotic administration. AFXD without evidence of amyloidosis was subsequently diagnosed.
获得性X因子缺乏症（AFXD）是一种非常罕见的凝血障碍。一名40岁无合并症的男性，因发热、全身不适及严重出血症状（包括大量血尿）而紧急入院。他的血小板计数正常，但凝血酶原时间和活化部分凝血活酶时间显著延长，这被认为是由于针对共同途径中凝血因子的自身抗体所致。尽管严重的大量血尿导致了短暂性肾衰竭，但他通过紧急免疫抑制治疗成功治愈。计算机断层扫描显示支气管肺炎，经抗生素治疗后病情改善。随后诊断为无淀粉样变性证据的AFXD。
