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埃及上埃及一家三级中心镰状细胞贫血患儿的循环微粒

Circulating Microparticles in Children With Sickle Cell Anemia in a Tertiary Center in Upper Egypt.

作者信息

Zahran Asmaa M, Elsayh Khalid I, Saad Khaled, Embaby Mostafa M, Youssef Mervat A M, Abdel-Raheem Yasser F, Sror Shaban M, Galal Shereen M, Hetta Helal F, Aboul-Khair Mohamed Diab, Alblihed Mohamd A, Elhoufey Amira

机构信息

1 Department of Clinical Pathology, South Egypt Cancer Institute, Assiut, Egypt.

2 Department of Pediatrics, Faculty of Medicine, Assiut University, Assiut, Egypt.

出版信息

Clin Appl Thromb Hemost. 2019 Jan-Dec;25:1076029619828839. doi: 10.1177/1076029619828839.

Abstract

Sickle cell disease (SCD) is a genetically inherited hemolytic anemia increasingly appreciated as a chronic inflammatory condition and hypercoagulable state with high thrombotic risk. It is associated with disturbed immune phenotype and function and circulating microparticles (MPs) derived from multiple cell sources. This study was carried out to determine MPs profiles in patients with sickle cell anemia (either on hydroxyurea (HU) therapy or those with no disease-modifying therapy) and to compare these profiles with healthy children. Moreover, our study assesses the potential impact of HU on other aspects of circulating MPs. We performed a cross-sectional study on 30 pediatric patients with SCD divided by treatment into 2 groups (those receiving HU or no therapy) attending Hematology Clinic and 20 age-matched healthy children. The blood samples obtained were analyzed for MPs by flow cytometry. Sickle cell disease group with no therapy showed elevated levels of total, platelet, and erythroid MPs. In contrast, therapy with HU was associated with normalization of MPs. This study provided additional evidence that HU is an effective treatment option in pediatric patients with SCD, as it seems that it decreases the abnormally elevated MPs in those patients.

摘要

镰状细胞病(SCD)是一种遗传性溶血性贫血,越来越被认为是一种慢性炎症性疾病和具有高血栓形成风险的高凝状态。它与免疫表型和功能紊乱以及源自多种细胞来源的循环微粒(MPs)有关。本研究旨在确定镰状细胞贫血患者(接受羟基脲(HU)治疗或未接受疾病改善治疗的患者)的MPs谱,并将这些谱与健康儿童进行比较。此外,我们的研究评估了HU对循环MPs其他方面的潜在影响。我们对30名儿科镰状细胞病患者进行了一项横断面研究,根据治疗情况将其分为2组(接受HU治疗或未接受治疗),这些患者在血液科门诊就诊,同时选取了20名年龄匹配的健康儿童。对采集的血样通过流式细胞术分析MPs。未接受治疗的镰状细胞病组显示总MPs、血小板MPs和红细胞MPs水平升高。相比之下,HU治疗与MPs正常化相关。本研究提供了额外的证据,表明HU是儿科镰状细胞病患者的一种有效治疗选择,因为它似乎降低了这些患者异常升高的MPs。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8cba/6714952/51f8ad5ca4f0/10.1177_1076029619828839-fig1.jpg

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