Kahn Marc J, McCrae Keith R
Division of Hematology-Oncology, Case Western Reserve University School of Medicine, 10900 Euclid Avenue, Cleveland, OH 44106, USA.
Curr Hematol Rep. 2004 Sep;3(5):317-23.
Immune thrombocytopenic purpura (ITP) is an uncommon disorder but a relatively frequent cause of isolated thrombocytopenia. The pathogenesis of ITP involves enhanced platelet clearance, primarily in the spleen, caused by antiplatelet glycoprotein antibodies. Most patients with ITP respond transiently to an initial course of corticosteroids, although only 20% of adult patients achieve a durable remission after such therapy. Classically, patients who fail corticosteroid therapy undergo splenectomy, with approximately two thirds of these individuals maintaining partial remissions 5 years later. However, due to evidence suggesting a continuous relapse rate in splenectomized patients with ITP, in addition to the emergence of newer agents associated with durable remissions in ITP patients with intact spleens, the therapeutic approach to ITP is evolving. This article reviews the role of splenectomy in patients with ITP and reviews new approaches to management of ITP in patients who fail an initial trial of corticosteroids.
免疫性血小板减少性紫癜(ITP)是一种罕见疾病,但却是孤立性血小板减少相对常见的病因。ITP的发病机制涉及抗血小板糖蛋白抗体导致的血小板清除增强,主要发生在脾脏。大多数ITP患者对初始疗程的皮质类固醇有短暂反应,尽管只有20%的成年患者经此类治疗后实现持久缓解。传统上,皮质类固醇治疗失败的患者会接受脾切除术,其中约三分之二的患者在5年后维持部分缓解。然而,由于有证据表明ITP脾切除患者存在持续复发率,此外出现了与脾脏完整的ITP患者持久缓解相关的新型药物,ITP的治疗方法正在不断演变。本文综述了脾切除术在ITP患者中的作用,并回顾了皮质类固醇初始试验失败的ITP患者的新管理方法。