Zimmer J, Andrès E, Noel E, Koumarianou A, Blicklé J-F, Maloisel F
Department of Onco-Hematology, Hopitaux Universitaires de strasbourg, Strasbourg, France.
Clin Lab Haematol. 2004 Apr;26(2):137-42. doi: 10.1111/j.1365-2257.2004.00591.x.
To define usefulness and response to therapy and outcome in adults with idiopathic thrombocytopenic purpura (ITP) in clinical practice. We retrospectively reviewed a cohort of 201 consecutive patients with ITP, diagnosed between 1985 and 1994. In particular, we analyzed the therapies used, their response rates, prognostic indicators of response and outcome. In 62 patients, with minor bleeding episodes and a mean (+/- SD) platelet count of 88 +/- 23 x 10(9)/l, no treatment was used and chronic ITP was diagnosed in 59%. A total of 139 patients, with bleeding episodes in 71.2% cases and a mean platelet count of 20 +/- 13 x 10(9)/l, received at least one treatment. Three patients died (1.5% of the series). Corticosteroids were used in 118 patients, with an initial response rate of 82.2% and a long-term complete response (CR) of only 22.9%. Intravenous immunoglobulin was used in 26 patients, with an initial transient response in more than 60%. A splenectomy was performed in 55 patients, with an initial response rate of 92.5% and a long-term CR in 60%. Young age and prior response to corticosteroids were significant predictors of a durable response to splenectomy. Danazol was given in 37 patients, with a favorable response in 73% of cases. Our results illustrate the guidelines of the American Society of Hematology. Patients with moderate thrombocytopenia do not require treatment. In severe cases, splenectomy is the only treatment giving durable cures in a significant proportion of patients. Despite frequent chronicity, ITP is life-threatening only in a minor subset of patients.
为明确成人特发性血小板减少性紫癜(ITP)在临床实践中的治疗有效性、反应及预后。我们回顾性分析了1985年至1994年间确诊的201例连续性ITP患者队列。具体而言,我们分析了所采用的治疗方法、其反应率、反应和预后的预测指标。62例患者有轻微出血发作,平均(±标准差)血小板计数为88±23×10⁹/L,未进行治疗,59%被诊断为慢性ITP。总共139例患者,71.2%有出血发作,平均血小板计数为20±13×10⁹/L,接受了至少一种治疗。3例患者死亡(占该系列的1.5%)。118例患者使用了皮质类固醇,初始反应率为82.2%,长期完全缓解(CR)仅为22.9%。26例患者使用了静脉注射免疫球蛋白,超过60%有初始短暂反应。55例患者进行了脾切除术,初始反应率为92.5%,长期CR为60%。年轻和既往对皮质类固醇有反应是脾切除术后持久反应的重要预测因素。37例患者使用了达那唑,73%的病例反应良好。我们的结果阐明了美国血液学会的指南。中度血小板减少的患者不需要治疗。在严重病例中,脾切除术是唯一能使相当比例患者获得持久治愈的治疗方法。尽管ITP常为慢性,但仅在一小部分患者中危及生命。