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Pathogenesis and Therapeutic Mechanisms in Immune Thrombocytopenia (ITP)

机译:免疫性血小板减少症(ITP)的发病机理和治疗机制

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摘要

Immune thrombocytopenia (ITP) is a complex autoimmune disease characterized by low platelet counts. The pathogenesis of ITP remains unclear although both antibody-mediated and/or T cell-mediated platelet destruction are key processes. In addition, impairment of T cells, cytokine imbalances, and the contribution of the bone marrow niche have now been recognized to be important. Treatment strategies are aimed at the restoration of platelet counts compatible with adequate hemostasis rather than achieving physiological platelet counts. The first line treatments focus on the inhibition of autoantibody production and platelet degradation, whereas second-line treatments include immunosuppressive drugs, such as Rituximab, and splenectomy. Finally, third-line treatments aim to stimulate platelet production by megakaryocytes. This review discusses the pathophysiology of ITP and how the different treatment modalities affect the pathogenic mechanisms.
机译:免疫性血小板减少症(ITP)是一种复杂的自身免疫性疾病,其血小板计数低。尽管抗体介导的和/或T细胞介导的血小板破坏都是关键过程,但ITP的发病机制仍不清楚。另外,现已认识到T细胞损伤,细胞因子失衡和骨髓生境的贡献是重要的。治疗策略旨在恢复具有足够止血功能的血小板计数,而不是实现生理血小板计数。一线治疗的重点是抑制自身抗体的产生和血小板的降解,而二线治疗包括免疫抑制药物,如利妥昔单抗和脾切除术。最后,第三线治疗旨在刺激巨核细胞产生血小板。这篇综述讨论了ITP的病理生理学以及不同的治疗方式如何影响其致病机制。

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