血管性血友病因子裂解蛋白酶活性及抑制物检测在获得性血栓性血小板减少性紫癜中的应用  

The value of the ADAMTS 13 activity and inhibitor measurements in Acquired Thrombotic Thrombocytopenic Purpura

作　　者：潘玉夏[1] 尚银涛[1] 王兴哲[1] 王茜[1] 温树鹏[1] 杨琳[1] 

机构地区：[1]河北医科大学第二医院血液内科河北省血液病重点实验室,河北石家庄050000

出　　处：《中国临床医生杂志》2015年第7期36-38,共3页Chinese Journal For Clinicians

摘　　要：目的探讨血管性血友病因子裂解蛋白酶（ADAMTS 13）活性及抑制物检测在获得性血栓性血小板减少性紫癜（TTP）中的应用价值。方法应用残余胶原结合实验检测2013年1月至2014年12月17例获得性TTP患者的ADAMTS 13活性及抑制物,结合临床资料分析其临床意义。结果 17例患者中包括男3例、女14例,中位年龄49（27-73）岁。全部患者用残余胶原结合实验检测ADAMTS 13活性均降低并伴有抑制物阳性,其中16例（94.1%）ADAMTS 13活性重度降低（〈10%）。17例患者中仅5例出现＂五联征＂,全部患者均有溶血性贫血及血小板减少,查乳酸脱氢酶（LDH）、间接胆红素（IBIL）增高。15例患者送检外周血涂片,10例发现破碎红细胞,6例〉1%。结论获得性TTP诊断缺乏特异性临床表现及实验室指标,残余胶原结合实验检测ADAMTS 13活性及抑制物,方法简便、结果可靠,ADAMTS 13活性重度降低,伴有抑制物阳性可以为获得性TTP的诊断与鉴别诊断提供重要的实验室依据,并为血浆置换联合免疫抑制治疗提供理论基础。Objective To investigate the value of the von Willebrand factor cleaving protease（ ADAMTS 13） activity and inhibitor measurements in acquired thrombotic thrombocytopenic purpura. Method Plasma ADAMTS 13 activity and inhibitor were measured by the residual collagen binding assay（ R-CBA）,17 acquired TTP patients admitted to the Second Hospital of Hebei Medical University from 2013 to 2014 were analyzed. Result Totally 17 acquired TTP patients included 3 males and 14 females,the median age was 49 years（ ranged 27-73）. Low ADAMTS 13 activity with the presence of an inhibitor had been demonstrated in all of the patients including a severely reduced ADAMTS13 activity（〈10%） in 16 patients. 5 patients had the classical pentad manifestations of TTP. Hemolyticanemia and thrombocytopenia appeared in all of the patients. The lactate dehydrogenase（ LDH） and the indirect bilirubin（ IBIL） were increased in all of the patients,and the schistocytes of peripheral blood smears were present in 10 cases（ 10 /15） including the percentage was greater than 1% in 6 cases. Conclusion The diagnosis of acquired TTP lack of specific clinical manifestations and laboratory indexes. A severely reduced ADAMTS 13 activity with the presence of an inhibitor confirms the diagnosis of acquired TTP,and it may suggest the need to add immunosuppressive therapy.

关 键 词：紫癜 血栓性血小板减少性 血管性血友病因子裂解蛋白酶 

分 类 号：R552[医药卫生—血液循环系统疾病]