摘要
目的探讨获得性血友病A(AHA)的临床特点、诊断与治疗方法,以提高对本病的认识。方法对25例获得性血友病A患者的临床表现、实验室检查、诊治经过与疗效等临床资料进行分析。结果 25例患者中,男13例,女12例;年龄53.28±19.78岁;11例发病前无基础疾病及明确诱因,病程3 d^2年。临床表现除基础疾病表现外,均有不同程度的皮肤瘀斑、肌肉肿胀疼痛、牙龈渗血、血尿、血便、腹腔血肿等出血表现;实验室检查为重要的依据,表现为aPTT延长,为94.9±20.9秒,血浆混合试验aPTT不能被正常血浆纠正,FⅧ:C降低为4.5±6.7%,FⅧ抑制物滴度在22.4±24.3 Bu;以上病例治疗根据出血情况及基础疾病与全身状况,予止血、消除抗体措施,获得完全缓解19例,部分缓解5例,死亡1例。其中5例使用利妥昔单抗治疗的患者均获得完全缓解。获缓解的24例,按FⅧ抑制物滴度水平分为3组,其与出血的严重程度、aPTT、FⅧ:C、获得部分缓解的时间等因素进行分析,显示并无明确的相关性。上述病例经治疗后临床症状好转,FⅧ活性升高至50%时间为23.8±13.3 d,FⅧ活性恢复正常时间为86.2±76.8 d;FⅧ抑制物滴度减少50%的时间28±16.7 d,FⅧ抑制物滴度≤0.6 Bu或转阴时间156±230.5 d。结论以上病例经及早诊断、及时补充凝血因子与应用免疫抑制药物后,出血症状可明显减轻,病情缓解,但要FⅧ抗体转阴则需较长时间治疗及结合其他疗法,利妥昔单抗联合免疫治疗已取得较好疗效,值得进一步研究。
Objective To discuss the clinical features,diagnosis and treatment of acquired hemophilia A（ AHA）. Methods To analyse the clinical data of 25 cases of AHA with clinical manifestations,laboratory examination,diagnosis and curative effect. Results Of the 25 patients,13 were male,12 female; age 53. 28 ±19. 78 y; 11 cases hadn＇t basic disease and inducement before the onset of AHA,the course of AHA was 3 d～ 2 years. In addition to clinical manifestations of basic disease,The manifestations included different degrees of skin ecchymosis,muscle swelling and pain,gingival bleeding,hematuria,bloody stool,abdominal hematoma.Abnormal laboratory examination indexes： aPTT extension,94. 9 ± 20. 9 s; Plasma aPTT hybrid test cannot be corrected with normal plasma; FⅧ： C decreased,4. 5 ± 6. 7%; FⅧ inhibitor level was 22. 4 ± 24. 3 Bu. After hemostasis and removal of blood coagulation factor antibody measures,19 patients achieved complete remission,partial remission in 5 cases,1 cases of death. 5 patients achieved complete remission treated with rituximab. 24 cases of remission were divided into 3 groups,according to the FⅧ inhibitor titers. There was no clear correlation between the titers of the FⅧ inhibitor level with the severity of bleeding,aPTT,FⅧ： C,the time of partial remission. The clinical symptoms of above cases improved after treatment,the time of F Ⅷ levels Increased by 50% was 23. 8 ± 13. 3 d,the recovery time of FⅧ levels was 86. 2 ± 76. 8 d,the time of FⅧ inhibitor levels decreased by 50% was 28 ± 16. 7 d,the clearance time of FⅧ inhibitor was 156 ± 230. 5 d. Conclusion Patients with AHA may achieved remission by the early diagnosis,timely supplement of blood coagulation factor and application of immunosuppressive drugs,and the bleeding symptoms can be significantly reduced. But a long course of treatment and combination with other therapies are necessary to clear FⅧ inhibitor. Rituximab combined with immunotherapy has achieved good curative effect,and it is worth further study.
出处
《血栓与止血学》
2015年第5期277-281,共5页
Chinese Journal of Thrombosis and Hemostasis
关键词
获得性血友病A
临床表现
治疗
Acquired
Hemophilia A
Clinical manifestation
Treatment
