Abstract: X-linked lymphoproliferative disease type 1 is a X-linked recessive inherited disorder associated with EB virus infection. there is severe clinical manifestation with poor prognosis.Its mechanisms，clinical features，differential diagnosis and treatments will be discussed in this article.

Key words: X-linked lymphoproliferative disease type 1, child

摘要： X连锁淋巴增殖综合征-1型是X连锁隐性遗传性疾病，与EB病毒感染密切相关，临床表现严重，预后不良。该文就该病的发病机制、临床表现、鉴别诊断及治疗进行阐述。

关键词: X连锁淋巴增殖综合征-1型, 儿童