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血小板动力学评估在儿童慢性特发性血小板减少性紫癜中的预后价值

The Prognostic Value of Platelet Kinetics Assessment in Pediatric Chronic Idiopathic Thrombocytopenic Purpura.

作者信息

Igrutinovic Nebojsa, Pantovic Jelena, Markovic Bojana, Medovic Marija, Cekerevac Milica, Markovic Vladimir, Odalovic Strahinja, Knezevic Sanja, Vujic Ana, Mihajlovic Isidora, Stojadinovic Nevena, Knezevic Dragan, Urakovic Nina, Andrejevic Ivana, Ristic Gordana J, Slavkovic Vladimir, Andric Kristina, Medovic Rasa

机构信息

University Clinical Center Kragujevac, Zmaj Jovina 30, 34000 Kragujevac, Serbia.

Department of Internal Medicine, Faculty of Medical Sciences, University of Kragujevac, Svetozara Markovića 69, 34000 Kragujevac, Serbia.

出版信息

Diagnostics (Basel). 2025 Jul 16;15(14):1790. doi: 10.3390/diagnostics15141790.

DOI:10.3390/diagnostics15141790
PMID:40722539
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12293436/
Abstract

: The assessment of platelet kinetics (APK) is recommended for patients with chronic idiopathic thrombocytopenic purpura (chITP). The aim of this study was to examine the importance of APK as a prognostic instrument in the selection of therapy in children with chITP. Retrospective, observational research, which included chITP children who were treated and subjected to APK in Serbia for 25 years (total number was 152). In the acute phase of the disease, 15% of patients had life-threatening bleeding, 15% were asymptomatic, and there were no cases of fatal bleeding. Mean platelet life was 0.89 ± 0.47 days. A total of 45% of patients had normal platelet production, and 35% had very low production. Among the patients, 55% exhibited splenic platelet sequestration, 35% had mixed sequestration, and 10% showed hepatic platelet sequestration. Platelet lifespan and production indices were less reliable parameters, due to numerous contradictory results, especially when compared with the location of platelet sequestration. Distribution of bleeding and therapy-resistant patients was dominant with mixed sequestration. Good therapy responders had dominant splenic sequestration. In the chronic phase of the disease, initial therapy was sufficient for 40-45% of patients, while another 25% required second-line therapy, regardless of platelet sequestration location. A total of 25% percent of patients had undergone splenectomy, and all of them were in stable remission. The remaining 10%, which represented the most severe cases, required all available therapies, had equally mixed and liver sequestration, and splenectomy showed no effect. APK may be a determining factor for the selection of splenectomy as a therapeutic option in case of predominantly splenic sequestration. Although the platelet production index has been explored in several studies, its clinical relevance remains controversial. In our findings, it did not contribute to therapeutic decision-making and may even lead to misinterpretation. The factors distinguishing the minority of bleeding and therapy-resistant patients with similar laboratory profiles remain unclear.

摘要

对于慢性特发性血小板减少性紫癜(chITP)患者,建议进行血小板动力学评估(APK)。本研究的目的是探讨APK作为预测工具在chITP儿童治疗方案选择中的重要性。这是一项回顾性观察研究,纳入了在塞尔维亚接受治疗并进行APK检测达25年的chITP儿童(总数为152例)。在疾病急性期,15%的患者有危及生命的出血,15%无症状,且无致命性出血病例。血小板平均寿命为0.89±0.47天。共有45%的患者血小板生成正常,35%的患者血小板生成极低。在这些患者中,55%表现为脾脏血小板扣押,35%为混合性扣押,10%表现为肝脏血小板扣押。血小板寿命和生成指数是不太可靠的参数,因为有许多相互矛盾的结果,尤其是与血小板扣押部位相比时。出血和治疗抵抗患者的分布以混合性扣押为主。治疗反应良好的患者以脾脏扣押为主。在疾病慢性期,40 - 45%的患者初始治疗就足够了,而另外25%的患者无论血小板扣押部位如何都需要二线治疗。共有25%的患者接受了脾切除术,且所有患者均处于稳定缓解状态。其余10%代表最严重的病例,需要所有可用的治疗方法,其扣押情况为混合性和肝脏性均等,脾切除术无效。对于以脾脏扣押为主的情况,APK可能是选择脾切除术作为治疗方案的决定性因素。尽管在多项研究中对血小板生成指数进行了探讨,但其临床相关性仍存在争议。在我们的研究结果中,它对治疗决策没有帮助,甚至可能导致误解。区分少数具有相似实验室检查结果的出血和治疗抵抗患者的因素仍不清楚。

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本文引用的文献

1
Update on the Use of Thrombopoietin-Receptor Agonists in Pediatrics.儿童患者中血小板生成素受体激动剂的应用进展。
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Splenectomy for immune thrombocytopenia: the evolution and preservation of treatment.免疫性血小板减少症的脾切除术:治疗的演变与保留
Haematologica. 2020 Nov 1;105(11):2507-2509. doi: 10.3324/haematol.2020.261099.
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Ann Hematol. 2020 Nov;99(11):2513-2520. doi: 10.1007/s00277-020-04257-2. Epub 2020 Sep 18.
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Br J Haematol. 2020 Jun;189(5):931-942. doi: 10.1111/bjh.16448. Epub 2020 Mar 4.
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