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成年原发性免疫性血小板减少症(ITP)患者的实际管理及对实践指南的遵循情况

Real-life management of primary immune thrombocytopenia (ITP) in adult patients and adherence to practice guidelines.

作者信息

Lozano María Luisa, Revilla N, Gonzalez-Lopez T J, Novelli S, González-Porras J R, Sánchez-Gonzalez B, Bermejo N, Pérez S, Lucas F J, Álvarez M T, Arilla M J, Perera M, do Nascimento J, Campos R M, Casado L F, Vicente V

机构信息

Hospital Morales Meseguer, IMIB-Arrixaca, Murcia, Spain.

Hospital Ramón y Cajal, Madrid, Spain.

出版信息

Ann Hematol. 2016 Jun;95(7):1089-98. doi: 10.1007/s00277-016-2665-3. Epub 2016 Apr 21.

Abstract

Very few data exist on the management of adult patients diagnosed with primary immune thrombocytopenia (ITP). The objectives of this study were to describe the diagnostic and treatment patterns for ITP and to compare the findings to recent ITP guidelines. We retrospectively analyzed the medical records of adult ITP patients diagnosed with primary ITP between January 2011 and June 2012 and examined whether management strategies were consistent or not with eight recent guideline-recommended practices. Overall, median age at the diagnosis of the disease (n = 101) was 58 years and median platelet count 12 × 10(9)/L with 75.2 % of patients having symptoms of ITP. The study perceived two major shortcomings in the diagnostic approach: (1) failure to perform peripheral blood film examination in 22.8 % of patients, a test that is mandatory by all guidelines, and (2) ordinary bone marrow assessment in more than half of the patients at diagnosis (50.5 %), a test not routinely recommended by guidelines. Low appropriateness in therapeutic management of patients included (1) unjustified use of intravenous immunoglobulin in the absence of bleeding in 54.8 % of patients and (2) splenectomy not being deferred until 6-12 months from diagnosis (median 161 days). Data also reflect a trend towards the early use of thrombopoietin receptor agonists in the treatment of patients who are refractory to any first-line therapy. We have recognized important areas of inapropriateness in the diagnostic and therapeutic management of adult ITP patients. Compliance with established guidelines should be encouraged in order to improve patient outcomes.

摘要

关于成年原发性免疫性血小板减少症(ITP)患者的管理,现有数据非常少。本研究的目的是描述ITP的诊断和治疗模式,并将研究结果与近期的ITP指南进行比较。我们回顾性分析了2011年1月至2012年6月期间诊断为原发性ITP的成年ITP患者的病历,并检查管理策略是否与近期八项指南推荐的做法一致。总体而言,确诊疾病时(n = 101)的中位年龄为58岁,中位血小板计数为12×10⁹/L,75.2%的患者有ITP症状。该研究发现诊断方法存在两个主要缺陷:(1)22.8%的患者未进行外周血涂片检查,而这是所有指南都要求进行的检查;(2)超过一半(50.5%)的患者在诊断时进行了普通骨髓评估,而这并非指南常规推荐的检查。患者治疗管理的合理性较低,包括:(1)54.8%的患者在无出血情况下不合理使用静脉注射免疫球蛋白;(2)脾切除术未推迟至诊断后6 - 12个月(中位时间为161天)。数据还反映出在治疗对任何一线治疗均无效的患者时,有早期使用血小板生成素受体激动剂的趋势。我们已经认识到成年ITP患者诊断和治疗管理中存在的重要不合理领域。应鼓励遵循既定指南,以改善患者预后。

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