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骨髓增生异常综合征患者的血小板表型异常。

Abnormal platelet phenotypes in patients with myelodysplastic syndromes.

机构信息

Department of Hematology, General Hospital, Tianjin Medical University, Tianjin, China.

出版信息

Int J Lab Hematol. 2020 Aug;42(4):371-379. doi: 10.1111/ijlh.13191. Epub 2020 Mar 23.

DOI:10.1111/ijlh.13191
PMID:32202052
Abstract

INTRODUCTION

Hemorrhage and infection are two main causes of death in patients with myelodysplastic syndromes (MDS), and it is becoming increasingly clear that platelet dysfunction can also affect the process of hemostasis and anti-infection. The aim of this study was to evaluate activation function and immune-related function of platelets in MDS.

METHODS

We included 29 MDS patients and divided them into different subgroups (low-risk group and high-risk group; untreated group and treated group; pretransfusion group and post-transfusion group) according to IPSS-R score, hypomethylating agents (HMAs) therapy, and platelet transfusion history. Platelet light scatter properties, expression of CD41a, activation-associated phenotypes (CD62p and CD63), and immune-associated phenotypes (CD154 and TLR4) were detected by multiparameter flow cytometry.

RESULTS

Expression of CD41a was decreased (P < .05), and no difference was found in platelet light scatter properties between MDS patients and healthy subjects (P > .05). Significantly decreased expression frequency and intensity of activation phenotype CD63 were found in patients with MDS (P < .05). Low-risk MDS showed lower expression frequency while high-risk MDS showed reduced mean fluorescence intensity (MFI) of CD63. Decreased expression of CD154 and TLR4 was found in MDS patients (P < .05) which was significantly elevated after HMAs therapy (P < .05). Particularly, MFI of CD154 and TLR4 reduced in high-risk MDS patients (P < .05).

CONCLUSION

Myelodysplastic syndromes patients displayed defective expression of both activation- and immune-associated platelet phenotypes, with differential mechanisms between low-risk and high-risk groups regarding phenotype alterations. The findings confirmed impaired platelet phenotypes in MDS which may assist in the diagnosis and identification of MDS patients.

摘要

简介

出血和感染是骨髓增生异常综合征(MDS)患者死亡的两个主要原因,越来越明显的是,血小板功能障碍也会影响止血和抗感染过程。本研究旨在评估 MDS 患者血小板的激活功能和免疫相关功能。

方法

我们纳入了 29 名 MDS 患者,并根据 IPSS-R 评分、低甲基化剂(HMAs)治疗和血小板输注史将其分为不同亚组(低危组和高危组;未治疗组和治疗组;输血前组和输血后组)。通过多参数流式细胞术检测血小板光散射特性、CD41a 表达、激活相关表型(CD62p 和 CD63)和免疫相关表型(CD154 和 TLR4)。

结果

与健康受试者相比,MDS 患者的 CD41a 表达降低(P<.05),但血小板光散射特性无差异(P>.05)。MDS 患者的激活表型 CD63 表达频率和强度明显降低(P<.05)。低危 MDS 表达频率较低,高危 MDS 则表现为 CD63 的平均荧光强度(MFI)降低。MDS 患者 CD154 和 TLR4 的表达降低(P<.05),经 HMAs 治疗后明显升高(P<.05)。特别是高危 MDS 患者的 CD154 和 TLR4 的 MFI 降低(P<.05)。

结论

骨髓增生异常综合征患者的激活和免疫相关血小板表型表达缺陷,低危和高危组之间的表型改变存在不同的机制。这些发现证实了 MDS 患者血小板表型受损,可能有助于 MDS 的诊断和识别。

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