Research Progress of Desialylation in Immune Thrombocytopenia -Review.
10.7534/j.issn.1009-2137.2018.03.050
- Author:
Jing-Yao MA
1
;
Zhen-Ping CHEN
1
;
Hao GU
1
;
Ling-Ling FU
1
;
Run-Hui WU
2
Author Information
1. 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.
2. 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: wurunhui@bch.com.cn.
- Publication Type:Journal Article
- MeSH:
Animals;
Antibodies;
Blood Platelets;
Humans;
Phagocytosis;
Thrombocytopenia
- From:
Journal of Experimental Hematology
2018;26(3):928-932
- CountryChina
- Language:Chinese
-
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.
