Zufferey Anne, Kapur Rick, Semple John W
Keenan Research Centre for Biomedical Science, St. Michael's Hospital, Toronto, ON M5B 1W8, Canada.
The Toronto Platelet Immunobiology Group, St. Michael's Hospital, Toronto, ON M5B 1W8, Canada.
J Clin Med. 2017 Feb 9;6(2):16. doi: 10.3390/jcm6020016.
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, thirdline 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.
免疫性血小板减少症（ITP）是一种以血小板计数低为特征的复杂自身免疫性疾病。尽管抗体介导和/或T细胞介导的血小板破坏是关键过程，但ITP的发病机制仍不清楚。此外，现在已经认识到T细胞损伤、细胞因子失衡以及骨髓微环境的作用也很重要。治疗策略旨在恢复与充分止血相适应的血小板计数，而不是达到生理血小板计数。一线治疗侧重于抑制自身抗体产生和血小板降解，而二线治疗包括免疫抑制药物，如利妥昔单抗，以及脾切除术。最后，三线治疗旨在刺激巨核细胞产生血小板。本综述讨论了ITP的病理生理学以及不同治疗方式如何影响致病机制。
