摘要
目的:血栓性血小板减少性紫癜(TTP)是一组急性、潜在和危及生命的血栓性微血管疾病。该病诊断主要通过其临床表现如血小板减少症、微血管病性溶血性贫血,此外,血管性血友病因子(vWF)的切割蛋白酶ADAMTS13活性低于10%是一项国际认可的诊断标准。临床上TTP的准确诊断和尽早的初始治疗能显著提高患者生存率。该病主要通过血浆输注或置换、糖皮质激素治疗以增加ADAMTS13活性;以及清除或抑制ADAMTS13抗体。抑制vWF-血小板相互作用、重组人ADAMTS13的应用,以及减少人中性粒细胞肽释放等措施,有望成为TTP治疗的新策略。本文就TTP的发病机制、诊断及治疗研究最新进展进行简要阐述。
Objective： Thrombotic thrombocytopenic purpura（TTP） is an acute, potentially life-threatening thrombotic microangiopathies（TMA）. TTP has mainly been diagnosed by clinical findings, such as thrombocytopenia and microangiopathic haemolytic anemia. In addition, the reduced activity of von Willebrand factor-cleaving metalloproteinase ADAMTS13 below 10% has been accepted internationally as a diagnostic criterion for TTP. In clinic,the accurate diagnosis and early initiation of therapy can significantly improve the survival rate of patients. Therapy should be focused on increasing ADAMTS13 activity and eliminating or inhibiting ADAMTS13 antibody by the infusion or exchange of therapeutic plasma and corticosteroids. Both the administration of recombinant ADAMTS13 and reducing the release of human neutrophil peptides（HNPs） would be novel promising strategies for the prevention of platelet-vWFinteraction.This review briefly summarizes the recent advances in terms of pathogenesis, diagnosis and treatment of TTP.
作者
贾茜
何杨
阮长耿
JIA Xi;HE Yang;RUAN Chang-Geng(Jiangsu Institute of Hematology,The First Affiliated Hospital of Soochow University,Suzhou 215006,Jiangsu Province,China.)
出处
《中国实验血液学杂志》
CAS
CSCD
北大核心
2018年第4期1230-1234,共5页
Journal of Experimental Hematology
