Statz A, Krueger G R, Roth B, Gladtke E
Eur J Pediatr. 1980;133(1):51-6. doi: 10.1007/BF00444755.
Immunopathological findings in a case with infantile X-linked agamma globulinemia (Bruton's disease) are reported and compared to similar data from two cousins of the patient. Besides a severe decrease in B-lymphocytes in blood and lymph node, residual B-cells showed an almost total defect in Ig surface receptor expression. In addition, there was an unusual lymphocyte population which apparently exhibited E-rosette formation (i.e. T-cell characteristics) and phagocytosis. The data suggest that in Bruton's disease there may be a disturbance of the T-cell system besides the well known B-cell deficiency.
报告了一例婴儿X连锁无丙种球蛋白血症(布鲁顿病)的免疫病理学发现,并与该患者的两个表亲的类似数据进行了比较。除了血液和淋巴结中的B淋巴细胞严重减少外,残留的B细胞在Ig表面受体表达上几乎完全缺陷。此外,存在一种异常的淋巴细胞群体,其明显表现出E花环形成(即T细胞特征)和吞噬作用。数据表明,在布鲁顿病中,除了众所周知的B细胞缺陷外,可能还存在T细胞系统的紊乱。