Sei Motoko, Mizuguchi Makiko, Yagi Hikaru, Kagawa Kumiko, Sekimoto Etsuko, Shibata Hironobu, Shigekiyo Toshio, Ozaki Shuji
Medical Education Center, Tokushima Prefectural Central Hospital.
Department of Hematology, Tokushima Prefectural Central Hospital.
Rinsho Ketsueki. 2022;63(7):733-739. doi: 10.11406/rinketsu.63.733.
Acquired factor V deficiency is a rare disease that presents with various bleeding symptoms because of the acquired production of factor V inhibitors and decrease in factor V activity. We have experienced five cases of acquired factor V deficiency diagnosed on the basis of abnormalities in coagulation tests in the last 10 years. All five patients were older men, of whom one had no bleeding symptoms, and three had a history of renal failure and malignant tumors. In the cross-mixing test, two of three cases demonstrated an inhibitor pattern, but one case showed a deficient pattern. In all cases, steroid treatment improved factor V activity as well as prothrombin time and activated partial thromboplastin time. However, patients with intracranial hemorrhage had a poor prognosis. Although this disease is rare, careful management is necessary, especially in the absence of bleeding symptoms and where cross-mixing test does not show an inhibitor pattern.
获得性因子V缺乏症是一种罕见疾病,由于获得性因子V抑制剂的产生和因子V活性降低而出现各种出血症状。在过去10年中,我们遇到了5例根据凝血试验异常诊断为获得性因子V缺乏症的病例。所有5例患者均为老年男性,其中1例无出血症状,3例有肾衰竭和恶性肿瘤病史。在交叉配血试验中,3例中有2例表现为抑制剂模式,但1例表现为缺乏模式。在所有病例中,类固醇治疗改善了因子V活性以及凝血酶原时间和活化部分凝血活酶时间。然而,颅内出血患者的预后较差。尽管这种疾病罕见,但仍需要仔细管理,尤其是在没有出血症状且交叉配血试验未显示抑制剂模式的情况下。