Imashuku Shinsaku, Hasegawa Takeshi, Kubo Kagekatsu, Nakato Masaki, Shima Midori
Division of Pediatrics and Hematology, Takasago-Seibu Hospital, Takasago, Hyogo.
Int Med Case Rep J. 2011 Apr 20;4:31-4. doi: 10.2147/IMCRJ.S19433. Print 2011.
Acquired anti-Factor V deficiency caused by inhibitor production is a rare coagulation disorder. Although this is a well known entity in the literature, choice of optimal treatment for an individual patient is difficult, given that no standard therapeutic measures are available because of rare incidence and various underlying diseases occurring in the elderly. An 88 year-old man treated for Hashimoto's disease was found to exhibit prolongation of both prothrombin time and activated partial thromboplastin time. Detailed study of coagulation factors revealed a deficiency of Factor V. Our patient's coagulation disorder resolved in two weeks with intravenous administration of prednisolone 20 mg/day. Clinical features of autoimmune disease-related Factor V deficiency are discussed, along with eight previously reported cases over the past 20 years.
由抑制剂产生导致的获得性抗凝血因子V缺乏是一种罕见的凝血障碍。尽管这在文献中是一个已知的实体,但由于其发病率低且老年患者存在各种基础疾病,没有标准的治疗措施,因此为个体患者选择最佳治疗方法很困难。一名接受Hashimoto病治疗的88岁男性被发现凝血酶原时间和活化部分凝血活酶时间均延长。对凝血因子的详细研究显示凝血因子V缺乏。我们的患者通过静脉注射20毫克/天的泼尼松龙,其凝血障碍在两周内得到缓解。本文讨论了自身免疫性疾病相关因子V缺乏的临床特征,并回顾了过去20年中先前报道的8例病例。