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免疫性血小板减少症脾切除术反应的预测因素:一项意大利多中心研究。

Predictors of Splenectomy Response in Immune Thrombocytopenia: A Multicentric Italian Study.

作者信息

Zoletto Simone, Pizzi Marco, De Crescenzo Andrea, Friziero Alberto, D'Amore Fabio, Carli Giuseppe, Vianelli Nicola, Auteri Giuseppe, Bertozzi Irene, Nichele Ilaria, Binotto Gianni, Dei Tos Angelo Paolo, Scarmozzino Federico, D'Amore Emanuele S G, Ceccato Jessica, Sabattini Elena, Cinetto Francesco, Piazza Francesco, Visentin Andrea, Zambello Renato, Trentin Livio, Vianello Fabrizio

机构信息

Hematology and Clinical Immunology Unit, Department of Medicine, University of Padua, 35122 Padua, Italy.

Surgical Pathology and Cytopathology Unit, Department of Medicine, University of Padua, 35122 Padua, Italy.

出版信息

J Clin Med. 2024 Dec 25;14(1):30. doi: 10.3390/jcm14010030.

DOI:10.3390/jcm14010030
PMID:39797114
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11722461/
Abstract

: Splenectomy leads to a high rate of remission in chronic primary immune thrombocytopenia (ITP), but its unpredictable long-term positive outcomes and that it is a irreversible surgical approach discourage clinicians and patients. The identification of predictors of response may redefine the timing of splenectomy. In this retrospective, multicentric study we aimed to investigate clinical-histological predictors of splenectomy response in ITP patients and provide an easy-to-use score to predict splenectomy response in ITP. : We considered a discovery set ( = 17) and a validation set ( = 30) of adult ITP patients, who underwent splenectomy for refractory disease in three Italian referral centers for ITP. : We found that the presence of autoimmune comorbidities, daily steroid dose prior to splenectomy, age at diagnosis and age at splenectomy were significantly associated with the outcome. Variables singly associated with an adverse outcome were combined into a clinical and a clinical-pathological score, allowing us to define a "high-risk" group which accounted for about 80% of the disease relapses observed in this cohort. At the same time, a certain clinical-pathological score indicated a "high-risk" group characterized by significantly poorer outcomes. Results were confirmed in the validation cohort. : An integrated set of clinical and histological parameters may predict the response to splenectomy in ITP patients. While these findings provide valuable insights, they were derived from a small cohort of patients and therefore require validation in larger, more diverse populations to ensure their generalizability and robustness.

摘要

脾切除术可使慢性原发性免疫性血小板减少症(ITP)的缓解率较高,但其不可预测的长期积极结果以及它是一种不可逆的手术方法,这使得临床医生和患者望而却步。确定反应的预测因素可能会重新定义脾切除术的时机。在这项回顾性多中心研究中,我们旨在调查ITP患者脾切除术反应的临床组织学预测因素,并提供一个易于使用的评分来预测ITP患者的脾切除术反应。我们纳入了一组发现集(n = 17)和一组验证集(n = 30)的成年ITP患者,这些患者在意大利三个ITP转诊中心因难治性疾病接受了脾切除术。我们发现自身免疫性合并症的存在、脾切除术前的每日类固醇剂量、诊断时的年龄和脾切除时的年龄与结果显著相关。与不良结果单独相关的变量被合并为一个临床评分和一个临床病理评分,这使我们能够定义一个“高危”组,该组约占该队列中观察到的疾病复发的80%。同时,某个临床病理评分表明存在一个结果明显较差的“高危”组。验证队列证实了结果。一组综合的临床和组织学参数可以预测ITP患者对脾切除术的反应。虽然这些发现提供了有价值的见解,但它们来自一小群患者,因此需要在更大、更多样化的人群中进行验证,以确保其普遍性和稳健性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/46bf/11722461/d0742d9d994d/jcm-14-00030-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/46bf/11722461/6d43d325a326/jcm-14-00030-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/46bf/11722461/3070db7f681a/jcm-14-00030-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/46bf/11722461/5f6479edc329/jcm-14-00030-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/46bf/11722461/d0742d9d994d/jcm-14-00030-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/46bf/11722461/6d43d325a326/jcm-14-00030-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/46bf/11722461/3070db7f681a/jcm-14-00030-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/46bf/11722461/5f6479edc329/jcm-14-00030-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/46bf/11722461/d0742d9d994d/jcm-14-00030-g004.jpg

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本文引用的文献

1
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Am J Blood Res. 2021 Aug 15;11(4):361-372. eCollection 2021.
2
Immune Thrombocytopenia: Recent Advances in Pathogenesis and Treatments.免疫性血小板减少症:发病机制与治疗的最新进展
Hemasphere. 2021 Jun 1;5(6):e574. doi: 10.1097/HS9.0000000000000574. eCollection 2021 Jun.
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Histology of the spleen in immune thrombocytopenia: clinical-pathological characterization and prognostic implications.免疫性血小板减少症的脾脏组织学:临床病理特征及预后意义。
Eur J Haematol. 2021 Feb;106(2):281-289. doi: 10.1111/ejh.13547. Epub 2020 Dec 2.
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American Society of Hematology 2019 guidelines for immune thrombocytopenia.美国血液学会 2019 年免疫性血小板减少症治疗指南。
Blood Adv. 2019 Dec 10;3(23):3829-3866. doi: 10.1182/bloodadvances.2019000966.
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Updated international consensus report on the investigation and management of primary immune thrombocytopenia.更新的原发性免疫性血小板减少症的调查和管理国际共识报告。
Blood Adv. 2019 Nov 26;3(22):3780-3817. doi: 10.1182/bloodadvances.2019000812.
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Thrombopoietin receptor agonists: ten years later.血小板生成素受体激动剂:十年后。
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