Immune thrombocytopenia: Pathophysiology and impacts of Romiplostim treatment

IF 6.9 2区医学 Q1 HEMATOLOGY

Blood Reviews Pub Date : 2024-06-20 DOI:10.1016/j.blre.2024.101222

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Abstract

Immune thrombocytopenia (ITP) is an autoimmune bleeding disease caused by immune-mediated platelet destruction and decreased platelet production. ITP is characterized by an isolated thrombocytopenia (<100 × 10⁹/L) and increased risk of bleeding. The disease has a complex pathophysiology wherein immune tolerance breakdown leads to platelet and megakaryocyte destruction. Therapeutics such as corticosteroids, intravenous immunoglobulins (IVIg), rituximab, and thrombopoietin receptor agonists (TPO-RAs) aim to increase platelet counts to prevent hemorrhage and increase quality of life. TPO-RAs act via stimulation of TPO receptors on megakaryocytes to directly stimulate platelet production. Romiplostim is a TPO-RA that has become a mainstay in the treatment of ITP. Treatment significantly increases megakaryocyte maturation and growth leading to improved platelet production and it has recently been shown to have additional immunomodulatory effects in treated patients. This review will highlight the complex pathophysiology of ITP and discuss the usage of Romiplostim in ITP and its ability to potentially immunomodulate autoimmunity.

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免疫性血小板减少症：病理生理学和罗米波司汀治疗的影响。

免疫性血小板减少症（ITP）是一种由免疫介导的血小板破坏和血小板生成减少引起的自身免疫性出血性疾病。免疫性血小板减少症的特点是孤立的血小板减少（9/L）和出血风险增加。该病病理生理学复杂，免疫耐受破坏导致血小板和巨核细胞破坏。皮质类固醇、静脉注射免疫球蛋白（IVIg）、利妥昔单抗和血小板生成素受体激动剂（TPO-RA）等治疗药物旨在增加血小板数量，防止出血并提高生活质量。TPO-RAs 通过刺激巨核细胞上的 TPO 受体直接刺激血小板生成。罗米洛司汀是一种 TPO-RA，已成为治疗 ITP 的主要药物。治疗可明显增加巨核细胞的成熟和生长，从而改善血小板的生成。本综述将重点介绍 ITP 复杂的病理生理学，并讨论 Romiplostim 在 ITP 中的应用及其潜在的免疫调节自身免疫的能力。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Blood Reviews 医学-血液学

CiteScore

13.80

自引率

1.40%

发文量

期刊介绍： Blood Reviews, a highly regarded international journal, serves as a vital information hub, offering comprehensive evaluations of clinical practices and research insights from esteemed experts. Specially commissioned, peer-reviewed articles authored by leading researchers and practitioners ensure extensive global coverage across all sub-specialties of hematology.

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