Abnormal distribution of distinct lymphocyte subsets in children with Wiskott-Aldrich syndrome

作者：Zhou, Lina; Li, Wenyan; Zhang, Xuan; Liu, Dawei; Ding, Yuan; Dai, Rongxin; Zhao, Xiaodong^*

来源：Human Immunology, 2017, 78(9): 565-573.

DOI：10.1016/j.humimm.2017.05.004

• Wiskott-Aldrich syndrome
• Lymphocyte subsets
• Immunophenotype
• Prognostic significance

Wiskott-Aldrich syndrome (WAS) is a severe and rare primary immunodeficiency. Several studies show that WAS protein (WASp) plays a key role in the function of certain lymphocyte subsets. So far, no study has described distinct immunophenotypic abnormalities associated with WAS; thus the prognostic significance of any such abnormalities is unclear. This study examined many differences in the percentage/absolute numbers of distinct lymphocyte subsets in 20 WAS patients and 20 age/sex-matched healthy controls, and analyzed the association between these abnormalities and clinical disease scores. The results showed that the numbers of CD4(+) T cells, B cells, and CD8(+) naive T cells were significantly lower in WAS patients; furthermore, the numbers in WASp-negative patients were lower than those in WASp-positive patients. WAS patients showed a selective reduction in expression of CD19 by naive and transitional B cells. There was a negative association between the number of B cells and the WAS clinical scores. Also, CD8(+) naive T cell numbers in patients with a score of 3-5A were lower than those in patients with a score of 2. The absence of WASp leads to a reduction in the population of specific lymphocyte subsets; therefore, these findings may help future management of patients with WAS.

• 出版日期2017-9
• 单位重庆医科大学