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BCR/ABL阴性骨髓增殖性肿瘤患者循环微颗粒水平升高——一项初步研究

High Level of Circulating Microparticles in Patients with BCR/ABL Negative Myeloproliferative Neoplasm - a Pilot Study.

作者信息

Aswad M H, Kissová J, Rihova L, Zavrelova J, Ovesná P, Penka M

出版信息

Klin Onkol. 2019 Spring;32(2):109-116. doi: 10.14735/amko2019109.

DOI:10.14735/amko2019109
PMID:30995850
Abstract

BACKGROUND

Microparticles (MPs) are small (0.1-1 μm) cell-derived vesicles released during activation or apoptosis, with a surface-exposed phosphatidylserine along with antigens indicating the cellular origin. The level of MPs is known to be elevated in thromboembolic diseases and malignancies; it is believed that MPs are not only amplifying but can also initiate the thrombogenesis processes. BCR/ABL negative myeloproliferative neoplasms (MPNs) are clonal haematopoietic diseases, which include polycythemia vera, essential thrombocythemia and primary myelofibrosis. One of the main problems of MPN patients is high risk and incidence of thrombosis which affect the survival, quality of life and life expectancy.

PATIENTS AND METHODS

The clinical significance of circulating MPs was assessed in a group of 179 patients with BCR/ABL-negative MPNs. Analysis of MPs was done using flow cytometry on 417 samples, and MPs procoagulation activity was performed using a functional assay called Zymuphen MP-activity (Hyphen Biomed, Neuville-sur-oise, France) on 274 samples.

RESULTS

Significantly higher absolute and relative count of platelet MPs was found in MPN patients when compared with healthy group, respectively (p = 0.001, p = 0.043). Erythrocyte MPs were also significantly higher in MPN patients than in the healthy group (p < 0.001). Procoagulation activity of MPs was as well significantly higher in patients compared to the control group (p < 0.001). Patients with primary myelofibrosis had decreased absolute and relative count of platelet MPs compared to polycythemia vera and essential thrombocythemia patients, respectively (p = 0.008, p = 0.014). Presence of JAK2V617F mutation was associated with higher absolute and relative count of platelet MPs, respectively (p = 0.045, p = 0.029).

CONCLUSION

Although some literature data support the hypothesis of a direct relation between MPs and thrombotic events in MPN patients, further studies are needed to evaluate the clinical implication of MPs in the hypercoagulation state of MPN patients.

摘要

背景

微粒(MPs)是在细胞激活或凋亡过程中释放的小(0.1 - 1μm)细胞衍生囊泡,其表面暴露有磷脂酰丝氨酸以及指示细胞来源的抗原。已知在血栓栓塞性疾病和恶性肿瘤中微粒水平会升高;据信微粒不仅会放大而且还能启动血栓形成过程。BCR/ABL阴性骨髓增殖性肿瘤(MPNs)是克隆性造血疾病,包括真性红细胞增多症、原发性血小板增多症和原发性骨髓纤维化。MPN患者的主要问题之一是血栓形成的高风险和发生率,这会影响患者的生存、生活质量和预期寿命。

患者和方法

在一组179例BCR/ABL阴性MPN患者中评估循环微粒的临床意义。使用流式细胞术对417份样本进行微粒分析,并使用一种名为Zymuphen MP-activity(Hyphen Biomed,法国努瓦西勒塞克)的功能测定法对274份样本进行微粒促凝活性检测。

结果

与健康组相比,MPN患者的血小板微粒绝对计数和相对计数分别显著更高(p = 0.001,p = 0.043)。MPN患者的红细胞微粒也显著高于健康组(p < 0.001)。与对照组相比,患者微粒的促凝活性也显著更高(p < 0.001)。与真性红细胞增多症和原发性血小板增多症患者相比,原发性骨髓纤维化患者的血小板微粒绝对计数和相对计数分别降低(p = 0.008,p = 0.014)。JAK2V617F突变的存在分别与血小板微粒的更高绝对计数和相对计数相关(p = 0.045,p = 0.029)。

结论

尽管一些文献数据支持MPs与MPN患者血栓形成事件之间存在直接关系的假设,但仍需要进一步研究来评估MPs在MPN患者高凝状态中的临床意义。

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