[Research Progress of Desialylation in Immune Thrombocytopenia -Review]

Jing-Yao Ma1, Zhen-Ping Chen1, Hao Gu1

  • 1National 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.

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.

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