2024 年第 9 期 第 0 卷

新生儿Fc受体及其抑制剂在原发免疫性血小板减少症中的研究进展

Research progress on neonatal Fc receptors and their inhibitors in primary immune thrombocytopenia

作者：朱赓达1房丽君1,2闫理想1范晨阳1孙慧1周欣丽1张禹成1史哲新1

英文作者：Zhu Gengda1 Fang Lijun12 Yan Lixiang1 Fan Chenyang1 Sun Hui1 Zhou Xinli1 Zhang Yucheng1 Shi Zhexin1

单位：1天津中医药大学第一附属医院血液科国家中医针灸临床医学研究中心，天津300381；2中国医学科学院血液病医院血液科国家血液系统疾病临床医学研究中心，天津300020

英文单位：1Department of Hematology First Teaching Hospital of Tianjin University of Traditional Chinese Medicine National Clinical Research Center for Chinese Medicine Acupuncture and Moxibustion Tianjin 300381 China; 2Department of Hematology Blood Diseases Hospital of the Chinese Academy of Medical Sciences National Clinical Medical Research Center for Blood Diseases Tianjin 300020 China

关键词：原发免疫性血小板减少症；新生儿Fc受体；新生儿Fc受体抑制剂；免疫球蛋白G

英文关键词：Primaryimmunethrombocytopenia;NeonatalFcreceptor;NeonatalFcreceptorinhibitors;ImmunoglobulinG

• 摘要：

• 原发免疫性血小板减少症(ITP)是一种由血小板自身抗体介导的自身免疫性疾病，大多数ITP患者具有免疫球蛋白G(IgG)亚型的抗血小板抗体，其通过与血小板和巨核细胞上糖蛋白的相互作用导致血小板破坏增加和抑制血小板的生成。新生儿Fc受体(FcRn)用于维持人体内IgG水平稳态。通过FcRn抑制剂靶向FcRn可以降低血液中的致病性IgG，证明了其对治疗ITP和其他自身免疫性疾病有效，并在一定程度上可改善ITP患者的血小板计数。efgartigimod、Rozanolixizumab均可与FcRn结合进而导致循环IgG减少。FcRn抑制剂还能间接延长罗米司亭的半衰期以提高治疗效果。本综述简要总结了ITP中血小板的破坏和生成抑制机制、FcRn和FcRn抑制剂作用的分子机制、FcRn抑制剂在ITP中的应用以及FcRn与罗米司亭、静脉注射Ig的联系。

• Primary Immune Thrombocytopenia(ITP) is an autoimmune disease mediated by platelet autoantibodies. Most ITP patients have antiplatelet antibodies against the immunoglobulin G (IgG) subtype, which interact with glycoproteins on platelets and megakaryocytes to increase platelet destruction and inhibit platelet production. Neonatal Fc receptor(FcRn) is used to maintain the homeostasis of IgG levels in the human body. Targeting FcRn by FcRn inhibitors can reduce pathogenic IgG in the blood, proving its efficacy in the treatment of ITP and other autoimmune diseases, and improving platelet counts in ITP patients to some extent. Both efgartigimod and Rozanolixizumab can bind to FcRn and lead to the reduction of circulating IgG. FcRn inhibitors can also indirectly prolong the half-life of romiplostim to improve the therapeutic effect. This review briefly summarizes the mechanism of inhibition of platelet destruction and production in ITP, the molecular mechanism of FcRn and FcRn inhibitors, the application of FcRn inhibitors in ITP, and the association of FcRn with romiplostim and intravenous Ig.