Aberrant Platelet Aggregation as Initial Presentation of Essential Thrombocythemia: Failure of Entero-Coated Aspirin to Reduce Platelet Hyperactivation
A. Morotti, C. Barale, Michele Sornatale, Emilia Giugliano, Vittorio Emanuele Muccio, C. Frascaroli, Marisa Pautasso, Alessandro Fornari, Isabella Russo
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引用次数: 0
Abstract
Essential thrombocythemia (ET) is a myeloproliferative neoplasm variant characterized by excessive production of platelets. Since the most common cause of mortality and morbidity in ET patients is thrombosis, the excessive production of platelets may cause thrombotic events. However, little is known about the function of platelets in ET. We report a female patient who presented as asymptomatic, without a remarkable medical history, and ET was diagnosed after an incidental finding of moderate thrombocytosis. Notably, together with thrombocytosis, an abnormal platelet phenotype was found for the presence of a massive, rapid and spontaneous formation of aggregates and platelet hypersensitivity to subthreshold concentrations of aggregating agonists. Bone marrow histopathological examination and genetic analysis with the JAK2 (V617F) gene mutation findings confirmed the initial suspicion of ET. Although the ET patient was placed on aspirin, the persistence of the platelet hyperactivation and hyperaggregability prompted a switch in antiplatelet medication from entero-coated (EC) to plain aspirin. As result, platelet hypersensitivity to agonists and spontaneous aggregation were no longer found. Collectively, our study demonstrates that platelet function analysis could be a reliable predictor of ET and that plain aspirin should be preferred over EC aspirin to attenuate platelet hyperreactivity.
原发性血小板增多症(ET)是一种骨髓增生性肿瘤变种,其特点是血小板生成过多。由于 ET 患者最常见的死亡和发病原因是血栓形成,因此血小板生成过多可能导致血栓事件。然而,人们对 ET 中血小板的功能知之甚少。我们报告了一名女性患者,她没有任何症状,也没有明显的病史,在偶然发现中度血小板增多后被诊断为 ET。值得注意的是,在发现血小板增多的同时,还发现了血小板表型异常,即存在大量、快速和自发形成的聚集体,以及血小板对亚阈浓度聚集激动剂的超敏反应。骨髓组织病理学检查和 JAK2(V617F)基因突变的遗传分析结果证实了最初对 ET 的怀疑。虽然 ET 患者服用了阿司匹林,但血小板高活性和高聚集性的持续存在促使患者将抗血小板药物从肠溶阿司匹林(EC)改为普通阿司匹林。结果,血小板不再对激动剂过敏,也不再出现自发性聚集。总之,我们的研究表明,血小板功能分析可作为 ET 的可靠预测指标,而且应首选普通阿司匹林而非 EC 阿司匹林来减轻血小板高反应性。
期刊介绍:
The International Journal of Molecular Sciences (ISSN 1422-0067) provides an advanced forum for chemistry, molecular physics (chemical physics and physical chemistry) and molecular biology. It publishes research articles, reviews, communications and short notes. Our aim is to encourage scientists to publish their theoretical and experimental results in as much detail as possible. Therefore, there is no restriction on the length of the papers or the number of electronics supplementary files. For articles with computational results, the full experimental details must be provided so that the results can be reproduced. Electronic files regarding the full details of the calculation and experimental procedure, if unable to be published in a normal way, can be deposited as supplementary material (including animated pictures, videos, interactive Excel sheets, software executables and others).