Kondo N, Motoyoshi F, Mori S, Kuwabara N, Orii T, German J
Department of Pediatrics, Gifu University School of Medicine, Japan.
Acta Paediatr. 1992 Jan;81(1):86-90. doi: 10.1111/j.1651-2227.1992.tb12088.x.
The immune state was evaluated over a 10-year period in two individuals with Bloom's syndrome. In both patients, serum concentrations of IgM were markedly low. Mildly decreased serum concentrations of IgG and IgA increased significantly with age, whereas the IgM levels remained low. From assessments of B-cell and T-cell functions in pokeweed mitogen-induced immunoglobulin production, the IgM deficiencies were thought to result from B-cell dysfunction. T-cell function appeared intact. Moreover, although the percentages of surface IgM-bearing cells were not reduced, the numbers of IgM-secreting cells were reduced. These findings suggest that the IgM deficiency is due to an abnormality in the maturation of surface IgM-bearing B cells into IgM-secreting cells.
对两名布卢姆综合征患者的免疫状态进行了为期10年的评估。在这两名患者中，血清IgM浓度均显著降低。血清IgG和IgA浓度轻度降低，并随年龄显著增加，而IgM水平仍较低。根据对商陆有丝分裂原诱导的免疫球蛋白产生中B细胞和T细胞功能的评估，IgM缺乏被认为是由B细胞功能障碍所致。T细胞功能似乎完好无损。此外，尽管表面带有IgM的细胞百分比没有降低，但分泌IgM的细胞数量减少。这些发现表明，IgM缺乏是由于表面带有IgM的B细胞成熟为分泌IgM的细胞过程中出现异常所致。
