去唾液酸化对免疫性血小板减少症影响的研究进展  

Research Progress of Desialylation in Immune Thrombocytopenia——Review

作　　者：马静瑶 陈振萍 谷昊 傅玲玲 吴润晖 MA Jing-Yao;CHEN Zhen-Ping;GU Hao;FU Ling-Ling;WU Run-Hui(National Center for Children＇s Healt;Capital Medical University, Beijing Children＇s Hospital, Hematology Oncology Center, Beijing Key Laboratory of Pediatric Hematology Oncolog;National Key Discipline of Pediatric;Key Laboratory of Major Diseases in Chil- dren, Ministry of Education, Beijing 100045, China)

机构地区：[1]国家儿童医学中心,首都医科大学附属北京儿童医院血液肿瘤中心,儿童血液病与肿瘤分子分型北京市重点实验室,儿科学国家重点学科,儿科重大疾病研究教育部重点实验室,北京100045

出　　处：《中国实验血液学杂志》2018年第3期928-932,共5页Journal of Experimental Hematology

基　　金：北京市自然科学基金课题(项目编号:7152053);北京市科技计划课题"首都临床特色应用研究"专项(课题编号:Z141107002514130);北京市医院管理局临床医学发展专项(项目编号:ZY201404)经费资助

摘　　要：免疫性血小板减少症(ITP)中一部分病人对常规一线治疗如肾上腺糖皮质激素、静脉注射丙种球蛋白(IVIG)等反应欠佳。近些年发现,这类病人存在FC-非依赖性血小板清除途径,主要是血小板表面糖蛋白发生去唾液酸化,最终被肝细胞表面的Ashwell-Morell受体(AMR)识别和吞噬,而不依赖于网状内皮系统FC受体介导的吞噬。各种原因引起的血小板神经氨酸酶(Neu1)表达上调均可使血小板表面糖蛋白发生去唾液酸化,如血小板的冷藏储存、败血症以及ITP等。目前发现,抗GPIbα抗体阳性的ITP接受一线治疗往往无效,推测抗GPIbα抗体可引起FC-非依赖性血小板清除。动物实验进一步证实抗GPIbα抗体可引起血小板去唾液酸化。有人尝试应用唾液酸酶抑制剂治疗此类ITP并获得了血小板的持续反应。本文结合血小板去唾液酸化的最新研究进展,对去唾液酸化在ITP发病机制和治疗方案选择中的作用作一综述。Some patients diagnosed as immune thrombocytopenia（ ITP） have poor response to common first-line therapy such as corticosteroid and immunoglobulin.Studies in recent years have found a FC-independent platelet clearance pathway exists,which is characterized by desialylation of platelet surface glycoprotein（ GP）,recognition and phagocytosis by Ashwell-Morell receptor（ AMR） on hepatocytes,independent on Fc receptors of the reticuloendothelial system.The up-regulation of neuraminidase-1（ Neu1） expression on platelet caused by various factors,such as cold storage of platelet,septicemia and ITP could desialylate GPs.It has been found that ITP with positive anti-GPIbα antibody mostly has a poor response to first-line therapy and indicated that such antibody may lead to FC-independent platelet clearance.It also has been proved that anti-GPIbα antibody could desialylate GPs on platelet in animal experiments.Researchers have tris to use sialidase inhibitor agent to treat ITP and got a persistent response of platelet.Here,the desialylation of platelet and its role in ITP pathogensis and therapy are review ed.

关 键 词：免疫性血小板减少症 去唾液酸化 唾液酸酶抑制剂 血小板特异性抗体 

分 类 号：R558.2[医药卫生—血液循环系统疾病]