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[去唾液酸化在免疫性血小板减少症中的研究进展——综述]

[Research Progress of Desialylation in Immune Thrombocytopenia -Review].

作者信息

Ma Jing-Yao, Chen Zhen-Ping, Gu Hao, Fu Ling-Ling, Wu Run-Hui

机构信息

National Center for Children's Health; Capital Medical University, Beijing Children's Hospital, Hematology Oncology Center, Beijing Key Laboratory of Pediatric Hematology Oncology; National Key Discipline of Pediatrics; Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing 100045, China.

National Center for Children's Health; Capital Medical University, Beijing Children's Hospital, Hematology Oncology Center, Beijing Key Laboratory of Pediatric Hematology Oncology; National Key Discipline of Pediatrics; Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing 100045, China. E-mail:

出版信息

Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2018 Jun;26(3):928-932. doi: 10.7534/j.issn.1009-2137.2018.03.050.

Abstract

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 reviewed.

摘要

一些被诊断为免疫性血小板减少症(ITP)的患者对常见的一线治疗,如皮质类固醇和免疫球蛋白反应不佳。近年来的研究发现存在一条不依赖Fc的血小板清除途径,其特征是血小板表面糖蛋白(GP)去唾液酸化,肝细胞上的阿什韦尔-莫雷尔受体(AMR)识别并吞噬,不依赖于网状内皮系统的Fc受体。各种因素,如血小板冷藏、败血症和ITP,导致血小板上神经氨酸酶-1(Neu1)表达上调,可使GP去唾液酸化。已发现抗GPIbα抗体阳性的ITP大多对一线治疗反应不佳,提示此类抗体可能导致不依赖Fc的血小板清除。动物实验也已证明抗GPIbα抗体可使血小板上的GP去唾液酸化。研究人员尝试使用唾液酸酶抑制剂治疗ITP,并获得了血小板的持续反应。在此,对血小板去唾液酸化及其在ITP发病机制和治疗中的作用进行综述。

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