text: 2.伴高IgM的免疫球蛋白缺乏症高IgM综合征（hyperIgMsyndrome，HM）70%为X连锁遗传，其余为常染色体隐性遗传；特点为B细胞内Ig转换障碍IgM正常或增高IgG、IgA和IgEX连锁高IgM血症因T细胞表面CD40配体基因突变，不能与B细胞表面CD40结合，使B细胞得不到活化刺激，是Ig转换障碍的原因。
entities.entity: 高IgM的免疫球蛋白缺乏症
entities.label: dis
entities.entity: 高IgM综合征
entities.label: dis
entities.entity: hyperIgMsyndrome
entities.label: dis
entities.entity: HM
entities.label: dis
entities.entity: X连锁
entities.label: bod
entities.entity: 常染色体
entities.label: bod
entities.entity: B细胞
entities.label: bod
entities.entity: Ig
entities.label: bod
entities.entity: B细胞内Ig转换障碍
entities.label: sym
entities.entity: IgM
entities.label: bod
entities.entity: IgM正常或增高
entities.label: sym
entities.entity: IgG
entities.label: bod
entities.entity: IgA
entities.label: bod
entities.entity: IgE
entities.label: bod
entities.entity: IgG、IgA和IgE
entities.label: sym
entities.entity: X连锁高IgM血症
entities.label: dis
entities.entity: T细胞表面CD40配体
entities.label: bod
entities.entity: B细胞表面CD40
entities.label: bod
entities.entity: B细胞
entities.label: bod
entities.entity: Ig
entities.label: bod