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原发性和继发性免疫性血小板减少症(ITP):是时候重新思考了。

Primary and secondary immune thrombocytopenia (ITP): Time for a rethink.

作者信息

González-López Tomás José, Provan Drew, Bárez Abelardo, Bernardo-Gutiérrez Angel, Bernat Silvia, Martínez-Carballeira Daniel, Jarque-Ramos Isidro, Soto Inmaculada, Jiménez-Bárcenas Reyes, Fernández-Fuertes Fernando

机构信息

Department of Hematology, Hospital Universitario de Burgos, Burgos, Spain.

Academic Haematology Unit, Blizard Institute, Barts and The London School of Medicine and Dentistry, Queen Mary University of London, London, UK.

出版信息

Blood Rev. 2023 Sep;61:101112. doi: 10.1016/j.blre.2023.101112. Epub 2023 Jun 29.

Abstract

There are not many publications that provide a holistic view of the management of primary and secondary ITP as a whole, reflecting the similarities and differences between the two. Given the lack of major clinical trials, we believe that comprehensive reviews are much needed to guide the diagnosis and treatment of ITP today. Therefore, our review addresses the contemporary diagnosis and treatment of ITP in adult patients. With respect to primary ITP we especially focus on establishing the management of ITP based on the different and successive lines of treatment. Life-threatening situations, "bridge therapy" to surgery or invasive procedures and refractory ITP are also comprehensively reviewed here. Secondary ITP is studied according to its pathogenesis by establishing three major differential groups: Immune Thrombocytopenia due to Central Defects, Immune Thrombocytopenia due to Blocked Differentiation and Immune Thrombocytopenia due to Defective Peripheral Immune Response. Here we provide an up-to-date snapshot of the current diagnosis and treatment of ITP, including a special interest in addressing rare causes of this disease in our daily clinical practice. The target population of this review is adult patients only and the target audience is medical professionals.

摘要

很少有出版物能从整体上全面介绍原发性和继发性免疫性血小板减少症(ITP)的管理情况,反映两者之间的异同。鉴于缺乏重大临床试验,我们认为当下非常需要全面综述来指导ITP的诊断和治疗。因此,我们的综述探讨了成年患者ITP的当代诊断和治疗。对于原发性ITP,我们特别关注基于不同且连续的治疗线来确立ITP的管理。危及生命的情况、手术或侵入性操作的“桥接治疗”以及难治性ITP在此也进行了全面综述。继发性ITP根据其发病机制分为三大鉴别组进行研究:中枢缺陷导致的免疫性血小板减少症、分化受阻导致的免疫性血小板减少症以及外周免疫反应缺陷导致的免疫性血小板减少症。在此我们提供了ITP当前诊断和治疗的最新概况,包括在日常临床实践中对该疾病罕见病因的特别关注。本综述的目标人群仅为成年患者,目标受众是医学专业人员。

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