获得性依赖VitK凝血因子缺乏症26例临床分析  

Clinical analysis of 26 patients with acquired deficiency of vitamin K-dependent coagulation factors

作　　者：陈海飞 金玲娟 吴天勤 唐杰庆 沈红石 李征洋 朱晶晶 秦龙梅 降宇峰 蒋文秀 余自强[2] 王兆钺[2] 

机构地区：[1]解放军苏州100医院血液科南京军区血液肿瘤治疗中心,江苏苏州215007 [2]苏州大学附属第一医院江苏省血液研究所

出　　处：《临床血液学杂志》2010年第4期409-411,415,共4页Journal of Clinical Hematology

基　　金：全军医药卫生科研基金项目(No:08MA019)

摘　　要：目的:探讨非婴幼儿获得性依赖VitK凝血因子缺乏症患者的临床特征及治疗方法。方法:回顾性分析在我院诊治的获得性依赖VitK凝血因子缺乏症26例患者的病因、临床表现、治疗以及随访情况。结果:18例患者病因不明(A组),6例为杀鼠药中毒(B组),2例为华法令过量(C组)。所有患者均同时或先后出现皮肤、黏膜、皮下及内脏出血,首发最常见出血部位(症状)为血尿;由于失血,15例患者Hb<100g/L,最低仅为36g/L;患者自出血症状始至明确诊断中位时间为10(2～120)d,其中11例患者1～4个月才确诊,各组自出血症状始至确诊的时间差异无统计学意义(P=0.81);全组有15例患者误诊,就诊时所有患者活化部分凝血活酶时间(APTT)、凝血酶原时间(PT)、国际标准化比值(INR)均显著延长或升高,血小板计数、凝血酶时间(TT)纤维蛋白原(Fg)均正常,各组APTT、PT、INR值差异无统计学意义,除2例华法令过量的患者仅停用华法令外,其余24例患者静脉给予VitK1(20～60)mg/d滴注1～2d后出血停止,部分患者同时使用凝血酶原复合物或(和)血浆输注,复查凝血指标恢复正常,治疗后患者APTT、PT及INR值与治疗前相比均差异有统计学意义(均P<0.01),静脉使用VitK1时间为1～10个月。长期静脉给予VitK治疗者中位随访6(1～20)个月,无一例患者复发。结论:非婴幼儿获得性依赖VitK凝血因子缺乏症多数原因不明,容易误诊。首发出血最常见的症状(部位)为血尿,半数以上的患者可因失血并发贫血。不明原因及杀鼠药中毒者临床表现、治疗反应及预后类似,均需接受长疗程静脉VitK治疗,预后良好。Objective：To explore the clinical features and treatment protocol of patients with acquired deficiency of vitamin K-dependent coagulation factors.Method：Retrospective analysis was performed on the data of the etiological factors,clinical manifestations,laboratory examinations,diagnoses and treatments of 26 patients with acquired deficiency of vitamin K-dependent coagulation factors.Result：There were 18 patients with unknown causes（A subgroup）,6 patients with clear histories of anticoagulant rodenticide intoxication（B subgroup）and two with warfarin overdosage（C subgroup）.The presentations of hemorrhage in the patients were various.The most common hemorrhage was hematuria（13/26）,followed by skin and mucous membrane bleeding（12/26）.In fifteen patients,hemoglobin（Hb）level was less than 100 g/L due to blood loss.And there were no difference of Hb and the time from hemorrhage manifestation to definite diagnosis among three subgroups.Fifteen of 26 patients were misdiagnosed and the median time from hemorrhage manifestation to definite diagnosis was 10 days（range,2 days to 4 months）.Laboratory examinations showed normal platelet count,thrombin time and normal fibrinogen concentration,but prolonged plasma prothrombin time（PT）,activated partial prothrombin time（APTT）and international normalized ration（INR）.Patients in C subgroup only discontinued warfarin,while the rest patients received intravenous vitamin K_1 at a dose of 20 to 60 mg/d.The bleeding symptoms disappeared in 1 to 2 days after treatment and the Hb level increased dramatically.There were significant differences of PT,APTT and INR in the patients before and after treatment（P0.01）.The patients received long time administration of vitamin K in-travenously even when bleeding ceased and the APTT and PT were normal.Conclusion：The presentations of hemorrhage in the patients with acquired deficiency of vitamin K-dependent coagulation factors were various.The most common hemorrhage symptoms was hematuria.Vitamin K_1

关 键 词：VITK缺乏 出血 临床特征 治疗 

分 类 号：R591.49[医药卫生—内科学]