获得性血友病A的临床诊疗现状  

Clinical Diagnostic and Therapeutic Situation of Patients with Acquired Hemophilia A

作　　者：刘文海[1] 

机构地区：[1]北海市人民医院血液内科,广西北海536000

出　　处：《医学综述》2007年第18期1395-1397,共3页Medical Recapitulate

摘　　要：获得性血友病A是机体自发产生因子Ⅷ抗体而引起的一种凝血缺陷病,常出现严重致命性出血,病死率高。该病少见,好发于中老年及产后女性,常被延误诊断。其基础疾病主要包括自身免疫性疾病、恶性肿瘤、产后、手术或创伤、感染性疾病、药物因素等,无基础疾病者占27.27%～80%。无遗传性或家族性出血史,且血浆活化部分凝血酶原时间明显延长、血浆凝血因子Ⅷ活性降低,FⅧ抑制物测定阳性可确诊。主要治疗措施包括控制急性出血、清除或减少FⅧ抗体的产生以及治疗基础疾病等。现就近年来获得性血友病A的流行病学、作用机制、临床诊断与治疗的研究进展。Acquired hemophilia A is a kind of disease with deficiency in blood coagulation resulting from spontaneous FⅧ：Ab,which often results in fetal bleeding with high case fatality.This disease is scare disease favoring people in middle aged and elder as well as postpartum female,which often is misdiagnosed.Its basic diseases mainly includes autoimmune disease,malignant tumor,post partum,operation or trauma,infectious disease,drug factors and so on.Patients with this disease having no basic diseases is only occupying 27.27%～80%.Patients can have final diagnosis with prolonged APTT（activated partial thromboplastin time）,decreased activity of Ⅷ and positive detection of FⅧ inhibitor,while,without history of genetic or familial bleeding.Its main therapeutic methods includes in controlling acute bleeding,eradicating or reducing the genesis FVIII,treating basic diseases and so on.This article reviews some progresses of acquired hemophilia A abour its epidemiology,mechanism of action,clinical diagnosis and treatment.

关 键 词：获得性血友病A 诊断 治疗 

分 类 号：R554[医药卫生—血液循环系统疾病]