Research Progress of Desialylation in Immune Thrombocytopenia -Review.
10.7534/j.issn.1009-2137.2018.03.050
- Author:
Jing-Yao MA
1
,
2
,
3
,
4
;
Zhen-Ping CHEN
1
,
2
,
3
,
4
;
Hao GU
1
,
2
,
3
,
4
;
Ling-Ling FU
1
,
2
,
3
,
4
;
Run-Hui WU
1
,
2
,
3
,
5
Author Information
1. National Center for Children's Health
2. Capital Medical University, Beijing Children's Hospital, Hematology Oncology Center, Beijing Key Laboratory of Pediatric Hematology Oncology
3. National Key Discipline of Pediatrics
4. Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing 100045, China.
5. 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.
