（2）T和B细胞均缺如SCID（T-</sup>B-</sup>SCID）：①RAG-1/RAG-2缺陷致T-B-SCID为重组活化基因-1（RAG-1）或RAG-2突变所致，婴儿期即发病，外周血T和B细胞计数均明显减少但NK活性正常或升高11p13编码VDJ基因重组酶的RAG1/RAG2突变，使T细胞受体（TCR）和B细胞表面免疫球蛋白（SIg）的VDJ结构重组发生障碍，患者外周血T、B细胞均减少，患儿在生后2～3个月即发生严重的复发性感染。
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