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遗传性血色素沉着症患者捐献的红细胞成分的特性

Properties of donated red blood cell components from patients with hereditary hemochromatosis.

作者信息

Sut Caroline, Hamzeh-Cognasse Hind, Laradi Sandrine, Bost Vincent, Aubrège Christine, Acquart Sophie, Vignal Martine, Boutahar Nadia, Arthaud Charles Antoine, Ange Eyraud Marie, Pozzetto Bruno, Tiberghien Pierre, Garraud Olivier, Cognasse Fabrice

机构信息

Etablissement Français du Sang Rhône-Alpes-Auvergne.

Université de Lyon, GIMAP-EA3064.

出版信息

Transfusion. 2017 Jan;57(1):166-177. doi: 10.1111/trf.13890. Epub 2016 Nov 2.

Abstract

BACKGROUND

Red blood cells (RBCs) contain large amounts of iron, and periodic therapeutic phlebotomy is thus the main treatment for hereditary hemochromatosis (HH). However, the donation of therapeutic phlebotomy products from asymptomatic patients for transfusion purposes remains controversial. In this study, we compared the quality of RBCs obtained from HH patients with those of non-HH RBCs, within the allowed 42-day storage period.

STUDY DESIGN AND METHODS

RBCs were obtained from HH patient donors and random regular blood donors by whole blood collection. RBCs were stored for up to 42 days, according to national regulations and standard blood bank conditions in France. The following variables were assessed: hematologic and biochemical results, RBC membrane and soluble inflammatory markers, and the proinflammatory potential of HH RBC supernatant toward endothelial cells in an in vitro model.

RESULTS

There were no major differences between the two groups in terms of biophysical, biochemical, or soluble immunomodulatory factors. However, we observed small but significant differences in changes in RBC membrane proteins during storage, including increased phosphatidylserine expression and decreased hemolysis in HH compared with normal RBCs. However, there were no differences in terms of bioactivity of soluble immunomodulatory factors in the RBC supernatant during storage between HH and control donors, as determined by their effects on endothelial cells in vitro.

CONCLUSIONS

These in vitro studies suggest that RBCs from HH patients appear, while exhibiting subtle differences, to be suitable for transfusion purposes according to currently accepted criteria.

摘要

背景

红细胞(RBCs)含有大量铁,因此定期治疗性放血是遗传性血色素沉着症(HH)的主要治疗方法。然而,将无症状患者的治疗性放血产品用于输血目的仍存在争议。在本研究中,我们在允许的42天储存期内,比较了从HH患者获得的红细胞与非HH红细胞的质量。

研究设计与方法

通过全血采集从HH患者献血者和随机的常规献血者中获取红细胞。根据法国的国家法规和标准血库条件,将红细胞储存长达42天。评估了以下变量:血液学和生化结果、红细胞膜和可溶性炎症标志物,以及在体外模型中HH红细胞上清液对内皮细胞的促炎潜力。

结果

两组在生物物理、生化或可溶性免疫调节因子方面没有重大差异。然而,我们观察到储存期间红细胞膜蛋白变化存在微小但显著的差异,包括与正常红细胞相比,HH患者红细胞中磷脂酰丝氨酸表达增加和溶血减少。然而,通过体外对内皮细胞的影响测定,HH患者与对照献血者在储存期间红细胞上清液中可溶性免疫调节因子的生物活性方面没有差异。

结论

这些体外研究表明,HH患者的红细胞虽然存在细微差异,但根据目前公认的标准似乎适合用于输血目的。

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