Anand Kartik, Shows Ryan, Veillon Diana, Mills Glen, Munker Reinhold
Department of Hematology/Oncology, and Pathology, at the LSU Health Sciences Center in Shreveport, LA.
J La State Med Soc. 2015 Jan-Feb;167(1):21-4. Epub 2015 Feb 15.
Factor VII deficiency is one of the most common of rare bleeding disorders(1). This autosomal recessive disorder has a prevalence of 1:500,000 with geographic variations. Clinical manifestations vary from asymptomatic to severe mucocutaneous bleeding. According to the International Registry of Factor VII Deficiency (IRF7) epistaxis is the most common clinical manifestation. Gastrointestinal and central nervous system(CNS) bleeding are rare presentations.(2-4) We present here the case of a patient with life-threatening CNS bleeding who was found at the age of 58 years to have congenital factor VII deficiency.
凝血因子VII缺乏症是最常见的罕见出血性疾病之一（1）。这种常染色体隐性疾病的患病率为1:500,000，存在地域差异。临床表现从无症状到严重的黏膜皮肤出血不等。根据国际凝血因子VII缺乏症登记处（IRF7）的数据，鼻出血是最常见的临床表现。胃肠道和中枢神经系统（CNS）出血则较为罕见（2-4）。我们在此介绍一名58岁被发现患有先天性凝血因子VII缺乏症且出现危及生命的中枢神经系统出血的患者病例。
