text: 表17-4以抗体缺陷为主的免疫缺陷病续表注：XLA：X连锁无丙种球蛋白血症；CVID：常见变异型免疫缺陷病；Igk：免疫球蛋白κ链1.X连锁无丙种球蛋白血症（Xlinkedagammaglobulinaemia，XLA）本病又称Bruton病，因定位于Xq12.3-3上的Bruton酪氨酸激酶（Btk）基因缺失或突变，使B细胞发育受阻于原B细胞，极少成熟B细胞（CD20+</sup>，CD19+</sup>，SIg+</sup>B细胞少于2%）。
entities.entity: 抗体缺陷
entities.label: dis
entities.entity: 免疫缺陷病
entities.label: dis
entities.entity: XLA
entities.label: dis
entities.entity: X连锁无丙种球蛋白血症
entities.label: dis
entities.entity: CVID
entities.label: dis
entities.entity: 常见变异型免疫缺陷病
entities.label: dis
entities.entity: Igk
entities.label: dis
entities.entity: 免疫球蛋白κ链
entities.label: dis
entities.entity: X连锁无丙种球蛋白血症
entities.label: dis
entities.entity: Xlinkedagammaglobulinaemia
entities.label: dis
entities.entity: XLA
entities.label: dis
entities.entity: Bruton病
entities.label: dis
entities.entity: Xq12.3-3
entities.label: bod
entities.entity: Bruton酪氨酸激酶
entities.label: bod
entities.entity: Btk
entities.label: bod
entities.entity: B细胞
entities.label: bod
entities.entity: 原B细胞
entities.label: bod
entities.entity: 成熟B细胞
entities.label: bod