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骨髓移植后年轻β地中海贫血患者中表达磷脂酰丝氨酸的红细胞及其微粒和活化血小板的标准化水平。

Normalized levels of red blood cells expressing phosphatidylserine, their microparticles, and activated platelets in young patients with β-thalassemia following bone marrow transplantation.

作者信息

Klaihmon Phatchanat, Vimonpatranon Sinmanus, Noulsri Egarit, Lertthammakiat Surapong, Anurathapan Usanarat, Sirachainan Nongnuch, Hongeng Suradej, Pattanapanyasat Kovit

机构信息

Graduate Program in Immunology, Department of Immunology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.

Research Division, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, 10700, Thailand.

出版信息

Ann Hematol. 2017 Oct;96(10):1741-1747. doi: 10.1007/s00277-017-3070-2. Epub 2017 Jul 26.

Abstract

Bone marrow transplantation (BMT) serves as the only curative treatment for patients with β-thalassemia major; however, hemostatic changes have been observed in these BMT patients. Aggregability of thalassemic red blood cells (RBCs) and increased red blood cell-derived microparticles (RMPs) expressing phosphatidylserine (PS) are thought to participate in thromboembolic events by initially triggering platelet activation. To our knowledge, there has been no report providing quantitation of these circulating PS-expressing RBCs and RMPs in young β-thalassemia patients after BMT. Whole blood from each subject was fluorescently labeled to detect RBC markers (CD235a) and annexin-V together with the known number TruCount™ beads. PS-expressing RBCs, RMPs, and activated platelets were identified by flow cytometry. In our randomized study, we found the decreased levels of three aforementioned factors compared to levels in patients receiving regular blood transfusion (RT). This study showed that BMT in β-thalassemia patients decreases the levels of circulating PS-expressing RBCs, their MPs, and procoagulant platelets when compared to patients who received RT. Normalized levels of these coagulation markers may provide the supportive evidence of the effectiveness of BMT for curing thalassemia.

摘要

骨髓移植(BMT)是重型β地中海贫血患者唯一的治愈性治疗方法;然而,在这些接受BMT的患者中观察到了止血变化。地中海贫血红细胞(RBC)的聚集性以及表达磷脂酰丝氨酸(PS)的红细胞衍生微粒(RMP)增加,被认为通过最初触发血小板活化而参与血栓栓塞事件。据我们所知,尚无报告对年轻的β地中海贫血患者接受BMT后这些循环中表达PS的RBC和RMP进行定量。对每个受试者的全血进行荧光标记,以检测RBC标志物(CD235a)和膜联蛋白-V以及已知数量的TruCount™微珠。通过流式细胞术鉴定表达PS的RBC、RMP和活化血小板。在我们的随机研究中,我们发现与接受常规输血(RT)的患者相比,上述三种因素的水平降低。这项研究表明,与接受RT的患者相比,β地中海贫血患者进行BMT可降低循环中表达PS的RBC、其微粒和促凝血小板的水平。这些凝血标志物的标准化水平可能为BMT治愈地中海贫血的有效性提供支持证据。

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