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Good 综合征患者骨髓中前 B 细胞的发育。

Precursor B-cell development in bone marrow of Good syndrome patients.

机构信息

Dept. of Clinical Immunology, La Paz University Hospital, Lymphocyte Pathophysiology in Immunodeficiencies Group La Paz Institute for Health Research (IdiPAZ), Madrid, Spain.

Dept. of Immunology, Erasmus MC, University Medical Center Rotterdam, the Netherlands.

出版信息

Clin Immunol. 2019 Mar;200:39-42. doi: 10.1016/j.clim.2018.11.009. Epub 2019 Jan 25.

Abstract

Good syndrome is an immunodeficiency presenting with thymoma, hypogammaglobulinemia and almost absent B cells. To investigate the origin of the B-cell lymphopenia in these patients, we studied B cell differentiation in the bone marrow of Good syndrome patients. We found very low numbers of precursor B cells in bone marrow of Good syndrome patients and a differentiation arrest after the pro-B-cell stage; this is different from other agammaglobulinemia patients with a defect in pre B-cell receptor signaling.

摘要

Good 综合征是一种以胸腺瘤、低丙种球蛋白血症和几乎没有 B 细胞为特征的免疫缺陷病。为了研究这些患者 B 细胞减少的起源,我们研究了 Good 综合征患者骨髓中的 B 细胞分化。我们发现 Good 综合征患者骨髓中的前体 B 细胞数量非常少,并且在前 B 细胞阶段后出现分化阻滞;这与其他丙种球蛋白血症患者的前 B 细胞受体信号缺陷不同。

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