中国临床医学2017，Vol.24Issue(2)：260-264,5.DOI:10.12025/j.issn.1008-6358.2017.20170237
获得性凝血因子Ⅴ缺乏症的临床诊治
Clinical analysis of acquired coagulation factor Ⅴ deficiency
摘要
Abstract
Objective:To conclude the clinical experience of acquired factor Ⅴ deficiency (AFⅤD).Methods:Clinical data of 10 cases with acquired coagulation factor Ⅴ deficiency were analyzed retrospectively, the clinical features were analyzed and clinical experiences were concluded.Results:The 10 patients included 7 males and 3 females.Their ages varied from 51 to 71 with a median age of 60 years.No one had hereditary coagulation factor deficiency or family history.Patients with acquired coagulation factor Ⅴ deficiency had different manifestations, including spontaneously mucous bleeding, epistaxis, hematuresis, melena and encephalic bleeding.Laboratory test showed that all patients had prolonged prothrombin time (PT) and activated partial thromboplastin time (APTT), decreased FⅤ activity, positive results of inhibitor qualitative or quantitative test.They were all diagnosed with acquired coagulation factor Ⅴ deficiency.All patients had fresh frozen plasma transfusions and were treated with steroid and immunosuppressive agents.There were 5 of the 10 patients had a remission and stopped bleeding, 3 of the 10 patients had poor therapeutic effect with steroid and cyclophosphamide, but had effective result with rituximab, 2 of the 3 patients had a normal level of FⅤ activity after treatment of rituximab and stopped bleeding, 1 of the 3 patients needed maintenance treatment of low dose rituximab, 2 of the 10 patients died of encephalic bleeding.Conclusions:Acquired factor Ⅴ inhibitor is a rare coagulation disorder with variable clinical symptoms.Steroid, cyclophosphamide and rituximab may be effective to eliminate the inhibitors.关键词
获得性因子Ⅴ抑制物/因子Ⅴ缺乏/免疫抑制剂/糖皮质激素Key words
acquirefactoV inhibitor/factoV deficiency/immunosuppressivregimens/glucocorticoid分类
医药卫生引用本文复制引用
陈云飞,刘葳,刘晓帆,付荣凤,黄月婷,薛峰,张磊,杨仁池..获得性凝血因子Ⅴ缺乏症的临床诊治[J].中国临床医学,2017,24(2):260-264,5.基金项目
国家重点研发计划(2016YFC0901503),天津市自然基金面上项目(16JCYBJC26700),中国医学科学院医学与健康科技创新工程经费资助(2016-I2M-1-002).Supported by the National Key Research and Development Program of China(2016YFC0901503), Tianjin Municipal Science and Technology Commission Grant(16JCYBJC26700), and CAMS Innovation Fund for Medical Sciences(CIFMS,2016-I2M-1-002). （2016YFC0901503）
