Cines Douglas B, McMillan Robert
Department of Pathology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA.
Annu Rev Med. 2005;56:425-42. doi: 10.1146/annurev.med.56.082103.104644.
Idiopathic thrombocytopenic purpura (ITP) is a common hematologic disorder manifested by immune-mediated thrombocytopenia. The diagnosis remains one of exclusion, after other thrombocytopenic disorders are ruled out based on history, physical examination, and laboratory evaluation. The goal of treatment is to raise the platelet count into a hemostatically safe range. The disorder is usually chronic, although there is considerable variation in the clinical course and most patients eventually attain safe platelet counts off treatment. However, a subset of patients has severe disease refractory to all treatment modalities, which is associated with considerable morbidity and mortality. This article focuses on the management of primary ITP in adults. We discuss criteria for treatment, the roles of splenectomy and other treatment options along with their side effects, and the management of ITP during pregnancy.
特发性血小板减少性紫癜(ITP)是一种常见的血液系统疾病,表现为免疫介导的血小板减少。在根据病史、体格检查和实验室评估排除其他血小板减少性疾病后,诊断仍需排除其他可能性。治疗的目标是将血小板计数提高到止血安全范围。该疾病通常为慢性,尽管临床病程存在很大差异,且大多数患者最终在停止治疗后可达到安全的血小板计数。然而,一部分患者患有对所有治疗方式均难治的严重疾病,这与相当高的发病率和死亡率相关。本文重点关注成人原发性ITP的管理。我们讨论治疗标准、脾切除术和其他治疗选择的作用及其副作用,以及妊娠期ITP的管理。