LONG-TERM STUDY OF THE IMMUNODEFICIENCY OF BLOOMS SYNDROME

被引：28

作者：

KONDO, N ^{[1
]}

MOTOYOSHI, F ^{[1
]}

MORI, S ^{[1
]}

KUWABARA, N ^{[1
]}

ORII, T ^{[1
]}

GERMAN, J ^{[1
]}

机构：

[1] NEW YORK BLOOD CTR,HUMAN GENET LAB,NEW YORK,NY 10021

来源：

ACTA PAEDIATRICA | 1992年 / 81卷 / 01期

关键词：

D O I：

10.1111/j.1651-2227.1992.tb12088.x

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

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.

引用

页码：86 / 90

页数：5

共 12 条

[1] CONGENITAL TELANGIECTATIC ERYTHEMA RESEMBLING LUPUS ERYTHEMATOSUS IN DWARFS - PROBABLY A SYNDROME ENTITY [J].