Pathogenesis of chronic immune thrombocytopenic purpura
Pathogenesis of chronic immune thrombocytopenic purpura
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DOI：
10.1097/moh.0b013e3282ba5552
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发表时间：
2007-09-01
影响因子：
3.2
通讯作者：
McMillan, Robert
中科院分区：
文献类型：
--
作者：
Cines, Douglas B.;McMillan, Robert
本文综述了免疫性血小板减少性紫癜的病理生理学的最新进展，免疫性血小板减少性紫癜是一种针对细胞特异性糖蛋白（GPIIb-IIIa，GPIb-IX等）的自身抗体加速血小板破坏的疾病。血小板抗体也可能损害巨核细胞发育和血小板周转，促血小板生成素水平正常或仅适度增加，血小板生成的代偿性增加在许多患者中无效。患者可能表现出免疫调节受损，表现为辅助性T淋巴细胞增殖增加。患者的细胞毒性T淋巴细胞在体外可溶解血小板。如果细胞毒性T淋巴细胞也能够扰乱巨核细胞的功能，这种机制可能有助于受损的血小板生成。Fc γ-RIIIa基因的多态性可能与对某些形式的治疗的反应相关，并且类似的遗传方法可能有助于鉴定在其自然史和对各种干预的反应方面不同的患者亚群。抑制血小板生成和Fc γ受体及其他多态性将在阐明慢性免疫性疾病的发病机制和靶向治疗中发挥越来越重要的作用。血小板减少性紫癜。
Purpose of reviewThis article summarizes recent insights into the pathophysiology of immune thrombocytopenic purpura, a disorder in which autoantibodies against cell-specific glycoproteins (GPIIb-IIIa, GPIb-IX and others) accelerate platelet destruction.Recent findingsAutoantibodies are produced by a limited number of B-cell clones. Platelet antibodies may also impair megakaryocyte development and platelet turnover, thromobopoietin levels are normal or only modestly increased and a compensatory increase in platelet production is not effective in many patients. Patients may show impaired immune regulation manifested by increased proliferation of helper T lymphocytes. Cytotoxic T lymphocytes from patients can lyse platelets in vitro. If cytotoxic T lymphocytes are also capable of perturbing megakaryocyte function, this mechanism may contribute to impaired platelet production. Polymorphisms in the Fc gamma-RIIIa gene may correlate with response to certain forms of therapy and similar genetic approaches may help to identify subsets of patients that differ in their natural history and response to various interventions.SummaryBetter understanding of autoantibody development, inhibition of thrombopoiesis and Fc gamma receptor and other polymorphisms will assume increased importance in elucidating the pathogenesis and targeting treatment of chronic immune thrombocytopenic purpura.