B cells in patients with X-linked agammaglobulinemia.
B cells in patients with X-linked agammaglobulinemia.
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DOI：
10.4049/jimmunol.134.5.3070
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发表时间：
1985-05
影响因子：
4.4
通讯作者：
M. Conley
中科院分区：
文献类型：
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作者：
M. Conley
X连锁无丙种球蛋白血症(XLA)被描述为一种前B细胞无法分化为B细胞的疾病。然而，在这种疾病的患者中偶尔也能看到少量的B细胞。由于这些细胞的表型可能有助于确定XLA中缺陷的位置，因此使用免疫荧光染色技术来表征XLA患者中可以发现的B细胞。7例患者外周血中均可检测到表面IgM阳性B细胞。这些B细胞占淋巴细胞总数的比例很小(0.01%~0.3%，对照组为3.2%~13.7%)，并且与对照组B细胞的表型不同。表面Ig M染色较亮(p<0.001.0 1)，Ia染色较弱(p<0.0 1)。这种表型与小鼠未成熟B细胞的表型相似。超过80%的患者B细胞表达表面IGD，且均表达B细胞标志B1，但仅有35%的患者表达B细胞标志B2。这种B细胞标志物是C3d受体和Epstein-Barr病毒受体，在个体发育中比B1表达得晚，并且可以在80%以上的对照B细胞上检测到。所有B细胞均表达kappa或lambda轻链。这些结果表明XLA患者前B细胞向B细胞分化的缺陷不是绝对的。B细胞的不成熟表型还表明，在这种疾病中，B细胞的成熟可能在一个以上的分化阶段受到阻碍。
X-linked agammaglobulinemia (XLA) has been described as a disorder in which pre-B cells fail to differentiate into B cells. However, a small number of B cells have been seen occasionally in patients with this disorder. Because the phenotype of these cells might be helpful in defining the site of the defect in XLA, immunofluorescent staining techniques were used to characterize the B cells that can be found in patients with XLA. Surface IgM-positive B cells could be detected in the peripheral circulation of all seven patients studied. These B cells constituted a very small percentage of the total lymphocytes (0.01 to 0.3% compared with 3.2 to 13.7% in controls) and differed in phenotype from control B cells. They were much more brightly stained for surface IgM (p less than 0.001) and less brightly stained for Ia (p less than 0.01). This phenotype is similar to that described for immature B cells in the mouse. Over 80% of the patients' B cells expressed surface IgD, and all expressed the B cell marker B1, but only 35% expressed the B cell marker B2. This B cell marker, which is the C3d receptor and the Epstein-Barr virus receptor, is expressed later in ontogeny than B1 and can be detected on over 80% of control B cells. All B cells expressed either kappa or lambda light chain. These findings indicate that the defect in differentiation of pre-B cells into B cells is not absolute in patients with XLA. The immature phenotype of the B cells additionally suggests that there may be a block in the maturation of B cells at more than one stage of differentiation in this disorder.