主要组织相容性复合体II类缺陷患者的临床病程。
Clinical course of patients with major histocompatibility complex class II deficiency.
作者信息
Saleem M A, Arkwright P D, Davies E G, Cant A J, Veys P A
机构信息
The Hospital for Sick Children, Great Ormond St, London, WC1N 3JH, UK.
出版信息
Arch Dis Child. 2000 Oct;83(4):356-9. doi: 10.1136/adc.83.4.356.
Abstract
The clinical course of 10 children who have been diagnosed with major histocompatibility complex (MHC) class II deficiency (bare lymphocyte syndrome) in the UK over the past eight years is described. They have had a generally poor prognosis, with only two of the 10 still alive despite eight attempts at bone marrow transplantation in six patients. Overwhelming viral infection was the predominant cause of death. Alternative transplant strategies or novel therapies are required for these patients.
摘要
本文描述了过去八年中在英国被诊断为主要组织相容性复合体（MHC）II类缺陷（裸淋巴细胞综合征）的10名儿童的临床病程。他们的预后普遍较差，10名患者中只有2名仍然存活，尽管6名患者进行了8次骨髓移植尝试。严重的病毒感染是主要死因。这些患者需要替代移植策略或新的治疗方法。
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