Psaila Bethan, Bussel James B
Division of Pediatric Hematology-Oncology, Weill-Cornell Medical College of Cornell University, 515 East 71st Street, S-724, New York, NY 10021, USA.
Hematol Oncol Clin North Am. 2007 Aug;21(4):743-59, vii. doi: 10.1016/j.hoc.2007.06.007.
Immune thrombocytopenic purpura (ITP) is an autoantibody-mediated thrombocytopenic disorder in which accelerated destruction of platelets occurs; platelet production may also be impaired by these antibodies. ITP is characterized by mucocutaneous bleeding. Rarely, more severe hemorrhages, such as intracranial hemorrhage, may occur. Traditional therapies, such as steroids, immunoglobulin therapy, and splenectomy, generally reduce peripheral destruction of platelets. More recently, with a better understanding of the immunopathologic mechanisms underlying thrombocytopenia, several new treatments have been developed, including thrombopoietic agents, specific inhibitors of Fcgamma receptor (FcgammaR) signaling, and B-cell depletion therapies. This article outlines current understanding of the epidemiology, etiology, diagnosis, and treatment of ITP. The focus is on recent pathophysiologic insights and areas of potential controversy in which studies are ongoing.
免疫性血小板减少性紫癜（ITP）是一种自身抗体介导的血小板减少性疾病，其中血小板加速破坏；这些抗体也可能损害血小板生成。ITP的特征是皮肤黏膜出血。很少会发生更严重的出血，如颅内出血。传统疗法，如类固醇、免疫球蛋白治疗和脾切除术，通常会减少血小板的外周破坏。最近，随着对血小板减少症潜在免疫病理机制的更好理解，已经开发出几种新的治疗方法，包括促血小板生成剂、Fcγ受体（FcγR）信号传导的特异性抑制剂和B细胞清除疗法。本文概述了目前对ITP的流行病学、病因、诊断和治疗的理解。重点是最近的病理生理学见解以及正在进行研究的潜在争议领域。
