“X-linked agammaglobulinemia，XLA”的概念、定义、翻译、参考文献-科学参考

单词	X-linked agammaglobulinemia，XLA
释义	X-linked agammaglobulinemia，XLA Encyclopedia 医学原发性B细胞缺陷释 X-linked agammaglobulinemia，XLAX连锁无丙种球蛋白血症在B细胞分化成熟的早期，X染色体上Bruton酪氨酸激酶（Btk）基因突变或缺失，使前B细胞信号转导受阻，分化和成熟发生障碍，不能合成免疫球蛋白的原发性B细胞免疫缺陷病。又称布鲁顿无丙种球蛋白血症、Bruton病。是最常见的以抗体缺陷为主的原发性免疫缺陷病。
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