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Blood:CVID的非接纳骨髓微环境影响B细胞的早期发育

2020-03-11 QQY MedSci原创

中心点:造血干细胞移植可使B细胞固有缺陷的患者重获正常的B细胞增殖,但对于非接纳骨髓微环的患者,则不能恢复B细胞再增殖。

中心点:

B细胞发育动态的体外研究将B细胞固有缺陷与骨髓(BM)微环境缺陷区分开来.

BM生态位损伤可导致B细胞缺陷和常见变异型免疫缺陷病(CVID)。

摘要:

常见变异型免疫缺陷病(CVID)是一种以对感染、低丙球蛋白血症和免疫失调的易感性增加为特征的疾病。虽然CVID被认为是一种外周B细胞间室异常,但25%的患者的骨髓早期B细胞发育也有所损失。

鉴于造血干细胞移植后B细胞重建较差,研究人员推测部分患者的骨髓环境已经不适合B细胞发育。

通过在体外研究骨髓来源的CD34+细胞向未成熟B细胞分化是动力学,研究人员发现能借此区分B细胞固有缺陷患者和非接纳型骨髓环境患者。前者的未成熟B细胞不能发育,后者的CD34+细胞可在体外分化为未成熟B细胞,但在体内分化效率较低。

在另一组患者中,未确定的前体细胞不能支持B细胞在体外持续发育,表明前体细胞可能较少或已经耗竭。造血干细胞移植可使B细胞固有缺陷的患者重获正常的B细胞增殖,但对于非接纳骨髓微环的患者,则不能恢复B细胞再增殖。

本研究指出了骨髓生态位在CVID发病机制中的重要意义。

原始出处:

Arianna Troilo, et al.Non permissive bone marrow environment impairs early B-cell development in common variable immunodeficiency. Blood. March 10, 2020.

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    2020-04-01 w363522449
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    2020-03-13 neurowu
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