skewed distribution of circulating activated natural killer t (nkt) cells in patients with common variable immunodeficiency disorders (cvid)偏态分布的循环激活自然杀伤t细胞(nkt)患者常见的变量免疫缺陷疾病(cvid).pdf

Skewed Distribution of Circulating Activated Natural Killer T (NKT) Cells in Patients with Common Variable Immunodeficiency Disorders (CVID) 3 1 3 2 Karina I. Carvalho , Karina M. Melo , Fernanda R. Bruno , Jennifer E. Snyder-Cappione , Douglas F. 2 1 1,3 Nixon , Beatriz T. Costa-Carvalho , Esper G. Kallas * ˜ ˜ 1 Federal University of Sao Paulo, Sao Paulo, Brazil, 2 Division of Experimental Medicine, San Francisco General Hospital, University of California San Francisco, San ˜ ˜ Francisco, United States of America, 3 University of Sao Paulo, Sao Paulo, Brazil Abstract Common variable immunodeficiency disorder (CVID) is the commonest cause of primary antibody failure in adults and children, and characterized clinically by recurrent bacterial infections and autoimmune manifestations. Several innate immune defects have been described in CVID, but no study has yet investigated the frequency, phenotype or function of the key regulatory cell population, natural killer T (NKT) cells. We measured the frequencies and subsets of NKT cells in patients with CVID and compared these to healthy controls. Our results show a skewing of NKT cell subsets, with CD4+ NKT cells at higher frequencies, and CD8+ NKT cells at lower frequencies. However, these cells were highly activated and expression CD161. The NKT cells had a higher expression of CCR5 and concomitantly expression of CCR5+CD69+CXCR6