网状细胞发育不良是极少见的严重联合免疫缺陷病之一。其特点为先天性粒细胞缺乏、淋巴细胞减少、淋巴结与胸腺发育不全,缺乏细胞免疫与体液免疫功能。从1959年报告第1例起,本症已有8例以上的报告。全部病例均在生后数天或数周内死于无法控制的感染。1例患儿曾在无菌环境中生存了17周。作者用与患儿组织相容兄弟的异源性骨髓移植,治疗了 Reticular dysplasia is one of the rare severe combined immunodeficiency diseases. It is characterized by congenital agranulocytosis, lymphopenia, lymph node and thymus hypoplasia, lack of cellular and humoral immune function. From the first report in 1959, the disease has been reported in more than 8 cases. All cases died of uncontrollable infections within days or weeks of life. One patient had been in sterile condition for 17 weeks. The authors were treated with a heterologous bone marrow transplant that was compatible with pediatric tissue