获得性血友病A的临床分析  

Clinical analysis of acquired hemophilia A

作　　者：周云[1] 吴春农[1] 谢晓英[1] 曹燕 马丽[1] Zhou Yun;Wu Chunnong;Xie Xiaoying;Cao Yah;Ma Li(Department of Hematology,First People＇s Hospital of Liangshan Yi Autonomous Prefecture,Xichang 615000,Sichuan Province,China)

机构地区：[1]凉山彝族自治州第一人民医院血液科,四川西昌615000

出　　处：《国际输血及血液学杂志》2018年第4期285-292,共8页International Journal of Blood Transfusion and Hematology

摘　　要：目的 探讨获得性血友病（AH）A的病因、临床特点、治疗方法及疗效.方法 选择2007年6月至2016年5月,凉山彝族自治州第一人民医院血液科收治的4例AHA患者为研究对象.回顾性分析这4例AHA患者的临床特点、实验室检查结果、治疗方法及疗效等临床资料,并结合相关文献,探讨AHA诊治的研究进展.结果 ①4例患者均以皮肤淤斑及肌肉血肿为主要表现.患者1,四肢脉搏微弱,血压为80 mmHg/40 mmHg（1 mmHg=0.133 kPa）;血常规检查结果示,血红蛋白（Hb）水平为69 g/L;凝血功能分析结果示,活化部分凝血活酶时间（APTT）为82.8 s,凝血因子FⅧ活性＜1.0％,凝血因子FⅧ抑制物滴度为3.20 Bu/mL,凝血因子FⅨ活性为1.0％;风湿三项检测结果示,类风湿因子（RF）水平为219.0 U/L,C反应蛋白（CRP）水平为46.19 mg/L.患者2,四肢脉搏微弱,血压为80 mmHg/50 mmHg;血常规检查结果示,Hb水平为58 g/L;凝血功能分析结果示,APTT＞248.0 s,凝血因子FⅧ活性＜2.0％,凝血因子FⅧ抑制物滴度为5.50 Bu/mL,凝血因子FⅨ活性为4.5％,凝血因子FⅪ活性为13.6％,凝血因子FⅫ活性为7.0％,凝血因子FⅨ抑制物滴度为0.98 Bu/mL;免疫学检查结果示,抗干燥综合征（SS）A抗体（＋＋＋）、抗SSB抗体（＋＋＋）、抗RO52抗体（＋＋＋）,抗酸性核蛋白抗体（＋＋）,抗核抗体（ANA）滴度为1∶3 200.患者3,血压为104 mmHg/66 mmHg;血常规检查结果示,Hb水平为46 g/L;凝血功能分析结果示,APTT明显延长为104.4 s,凝血因子FⅧ活性为4.4％,FⅧ抑制物滴度为6.20 Bu/mL;免疫学检查结果示：ANA滴度为1∶3 200,抗SSA抗体（＋）,抗线粒体M2抗体（＋）,ACA（-）.患者4,血压为106 mmHg/72 mmHg;血常规检测结果示,白细胞计数为23.21×109/L,中性粒细胞比例为90％,Hb水平为58 g/L;凝血功能分析结果示,APTT为96.9s,凝血因子FⅧ活性为3.7％,凝血因子FⅧ抑制物滴度为3.30 Bu/mL;胸部CT结果示,右肺下叶外�Objective To investigate the etiology,clinical features,treatment and curative effect of acquired hemophilia （AH） A.Methods From June 2007 to May 2016,a total of 4 AHA patients who were admitted to Department of Hematology,First People＇s Hospital of Liangshan Yi Autonomous Prefecture were included in this study.The clinical characteristics,laboratory examination results,treatment methods and curative effect of the 4 AHA patients were analyzed retrospectively,and the research progress of diagnosis and treatment of AHA was discussed in combination with relevant literature.Results ① Skin stasis and muscle hematoma were the main manifestations in 4 AHA patients.Case 1 had a weak pulse in all extremities and a blood pressure of 80 mmHg/40 mmHg （1 mmHg =0.133 kPa）.Blood routine examination results showed that level of hemoglobin （Hb） was 69 g/L.Blood coagulation function analysisresults showed activated partial thromboplastin time （APTT） was 82.8 s,FⅧ activity〈1.0%,titer of FⅧ inhibitor was 3.20 Bu/mL,FⅨ activity was 1.0%.Rheumatoid arthritis （RA） test results showed level of rheumatoid factor （RF） was 219.0 U/L,and level of C reaction protein （CRP） was 46.19 mg/L.Case 2 had a weak pulse in all extremities and a blood pressure of 80 mmHg/50 mmHg.Blood routine examination results showed that the Hb level was 58 g/L.Blood coagulation function analysis results showed APTT〉 248.0 s,FⅧ activity〈2.0%,titer of FⅧ inhibitor was 5.50 Bu/mL,FⅨ activity was 4.5%,FⅪ activity was 13.6%,FⅫ activity was 7.0%,titer of FⅨ inhibitor was 0.98 Bu/mL.Immunological test showed Sjogrensyndrome （SS） antibody （＋＋＋）,SSB antibody （＋＋＋）,RO52 antibody （＋＋＋）,acidic nucleoprotein antibody （＋＋）,titer of antinuclear antibody （ANA） was 1 ∶ 3 200.Case 3 had a blood pressure of 104 mmHg/66 mmHg.Blood routine examination results showed that Hb level was 46 g/L.Blood coagulation function analysis results showed APTT was 104.4 s,FⅧ activity was 4.4%,tit

关 键 词：血友病A 因子Ⅷ 凝血因子 部分凝血活酶时间 疗法 血液输注 糖皮质激素 免疫抑制剂 获得性血友病A 

分 类 号：R554.1[医药卫生—血液循环系统疾病]