MD-PhD Program, Yale School of Medicine, New Haven, Connecticut.
Division of Hematology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Curr Opin Hematol. 2023 Nov 1;30(6):210-218. doi: 10.1097/MOH.0000000000000781. Epub 2023 Jul 27.
The platelet surface harbors a lush forest of glycans (carbohydrate polymers) attached to membrane proteins and lipids. Accumulating evidence suggests that these glycans may be relevant to the pathophysiology of immune thrombocytopenia (ITP). Here, we critically evaluate data that point to a possible role for loss of sialic acid in driving platelet clearance in ITP, comment on the potential use of neuraminidase inhibitors for treatment of ITP, and highlight open questions in this area.
Multiple lines of evidence suggest a role for loss of platelet sialic acid in the pathophysiology of thrombocytopenia. Recent work has tested the hypothesis that neuraminidase-mediated cleavage of platelet sialic acid may trigger clearance of platelets in ITP. Some clinical evidence supports efficacy of the viral neuraminidase inhibitor oseltamivir in ITP, which is surprising given its lack of activity against human neuraminidases.
Further study of platelet glycobiology in ITP is necessary to fill key knowledge gaps. A deeper understanding of the roles of platelet glycans in ITP pathophysiology will help to guide development of novel therapies.
血小板表面附着着丰富的糖链（碳水化合物聚合物），这些糖链附着在膜蛋白和脂质上。越来越多的证据表明，这些糖链可能与免疫性血小板减少症（ITP）的病理生理学有关。在这里，我们批判性地评估了指向糖链唾液酸化缺失可能导致 ITP 中血小板清除的相关数据，评论了神经氨酸酶抑制剂治疗 ITP 的潜在用途，并强调了该领域的开放性问题。
多项证据表明血小板唾液酸化缺失在血小板减少症的病理生理学中起作用。最近的工作已经检验了这样一个假设，即血小板唾液酸的神经氨酸酶介导的裂解可能触发 ITP 中的血小板清除。一些临床证据支持神经氨酸酶抑制剂奥司他韦治疗 ITP 的疗效，这令人惊讶，因为它对人类神经氨酸酶没有活性。
有必要在 ITP 中进一步研究血小板糖生物学，以填补关键的知识空白。更深入地了解血小板糖在 ITP 病理生理学中的作用将有助于指导新疗法的开发。
