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[免疫性血小板减少症的病理生理学与治疗的最新进展]

[Recent progress in immune thrombocytopenia pathophysiology and treatment].

作者信息

Kato Hisashi

机构信息

Department of Hematology and Oncology, Osaka University Graduate School of Medicine.

出版信息

Rinsho Ketsueki. 2023;64(9):1106-1115. doi: 10.11406/rinketsu.64.1106.

Abstract

Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by increased platelet destruction and impaired platelet production from bone marrow megakaryocytes. The details of different platelet destruction mechanisms resulting from differences in autoantibodies and autoantibody-independent direct platelet destruction remain unclear although antiplatelet autoantibodies directed against platelet glycoproteins, such as GPIIb/IIIa and GPIb, play a central role in ITP pathogenesis. ITP is diagnosed by excluding other causes of thrombocytopenia due to the lack of standard tests or biomarkers for its confirmation. Plasma thrombopoietin level measurement and reticulated platelet ratio are useful in distinguishing the cause of thrombocytopenia and will be included in the new "Diagnostic reference guide of adult immune thrombocytopenia." Currently, the treatment of refractory chronic ITP is mainly based on thrombopoietin receptor agonists, but ITP drugs with novel mechanisms of action are actively developed. New therapeutic agents are expected to be selected based on an accurate diagnosis and tailored to the pathophysiology of each case in ITP treatment.

摘要

免疫性血小板减少症(ITP)是一种自身免疫性疾病,其特征是血小板破坏增加以及骨髓巨核细胞产生血小板的功能受损。尽管针对血小板糖蛋白(如GPIIb/IIIa和GPIb)的抗血小板自身抗体在ITP发病机制中起核心作用,但由自身抗体差异和自身抗体非依赖性直接血小板破坏导致的不同血小板破坏机制的细节仍不清楚。由于缺乏用于确诊的标准检测方法或生物标志物,ITP通过排除其他血小板减少症病因来诊断。血浆血小板生成素水平测定和网织血小板比率有助于区分血小板减少症的病因,并将纳入新的“成人免疫性血小板减少症诊断参考指南”。目前,难治性慢性ITP的治疗主要基于血小板生成素受体激动剂,但具有新作用机制的ITP药物正在积极研发。在ITP治疗中,有望根据准确的诊断选择新的治疗药物,并针对每个病例的病理生理学进行个性化治疗。

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