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Reduced numbers of circulating group 2 innate lymphoid cells in patients with common variable immunodeficiency.
Eur J Immunol ; 47(11): 1959-1969, 2017 11.
Artigo em Inglês | MEDLINE | ID: mdl-28718914
ABSTRACT
Recent studies identified an emerging role of group 2 and 3 innate lymphoid cells (ILCs) as key players in the generation of T-dependent and T-independent antibody production. In this retrospective case-control study, CD117+ ILCs (including the majority of ILC2 and ILC3) were reduced in patients with common variable immunodeficiency (CVID). The reduction in CD117+ ILCs was distinctive to CVID and could not be observed in patients with X-linked agammaglobulinemia. Patients with a more pronounced reduction in CD117+ ILC numbers showed significantly lower numbers of peripheral MZ-like B cells and an increased prevalence of chronic, non-infectious enteropathy. Subsequent phenotyping of ILC subsets in CVID revealed that the reduction in CD117+ ILC numbers is due to a reduction in ILC2 numbers. In vitro expansion of CVID ILC2 in response to IL-2, IL-7, IL-25 and IL-33 was impaired. Furthermore, upregulation of MHCII and IL-2RA in response to IL-2, IL-7, IL-25 and IL-33 was impaired in CVID ILC2. Thus, our results indicate a dysregulation of ILC subsets with a reduction in ILC2 numbers in CVID, however, further studies are needed to explore whether ILC abnormalities are a primary finding or secondary to disease complications encountered in CVID.
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Texto completo: Disponível Coleções: Bases de dados internacionais Base de dados: MEDLINE Assunto principal: Subpopulações de Linfócitos / Imunodeficiência de Variável Comum Tipo de estudo: Estudo de casos e controles Limite: Feminino / Humanos / Masculino / Meia-Idade Idioma: Inglês Revista: Eur J Immunol Ano de publicação: 2017 Tipo de documento: Artigo País de afiliação: Áustria