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.
摘要：
免疫性血小板减少症（ITP）是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。ITP的特征是孤立的血小板减少症（<100×109/L）和出血风险增加。该疾病具有复杂的病理生理学,其中免疫耐受性破坏导致血小板和巨核细胞破坏。治疗如皮质类固醇，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）旨在增加血小板计数以防止出血并提高生活质量。TPO-RA通过刺激巨核细胞上的TPO受体来直接刺激血小板产生。Romiplostim是一种TPO-RA，已成为ITP治疗的中流砥柱。治疗显著增加巨核细胞成熟和生长,导致改善的血小板产生,并且最近已显示其在治疗的患者中具有额外的免疫调节作用。这篇综述将强调ITP的复杂病理生理学，并讨论Romiplostim在ITP中的用途及其潜在免疫调节自身免疫的能力。
