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。
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