原发免疫性疾病相关的淋巴增殖性疾病是指在原发免疫缺陷或原发免疫调节紊乱的疾病基础上发生的淋巴增殖性疾病(lymphoproliferative disease,LPD)。严重联合免疫缺陷症(severe combinedimmunodeficiency,SCI D),X连锁的淋巴增殖性疾病(X‐linked lym phoproliferative disorder,XLP),Nij megen断裂综合征(Nij megen breakage syndrome,NBS)。高IgM综合征(hyper‐IgM ......