Severely Impaired Control of Bacterial Infections in a Patient With Cystic Fibrosis Defective in Mucosal-Associated Invariant T Cells

Pincikova, TereziaUppsala universitet,Lung- allergi- och sömnforskning,Karolinska Univ Hosp Huddinge, Stockholm CF Ctr, Stockholm, Sweden;Karolinska Inst, Dept Clin Sci Intervent & Technol, Div Pediat, Stockholm, Sweden;Karolinska Univ Hosp Huddinge, Karolinska Inst, Ctr Infect Med, Dept Med, F59, S-14186 Stockholm, Sweden (författare)

Here we report a unique case of a patient with cystic fibrosis characterized by severely impaired control of bacterial respiratory infections. This patient's susceptibility to such infections was much worse than expected from a cystic fibrosis clinical perspective, and he died at age 22 years despite extensive efforts and massive use of antibiotics. We found that this severe condition was associated with a near-complete deficiency in circulating mucosal-associated invariant T (MAIT) cells as measured at several time points. MAIT cells are a large, recently described subset of T cells that recognize microbial riboflavin metabolites presented by the highly evolutionarily conserved MR1 molecules. The MAIT cell deficiency was specific; other T-cell subsets were intact. Even though this is only one unique case, the findings lend significant support to the emerging role of MAIT cells in mucosal immune defense and suggest that MAIT cells may significantly modify the clinical phenotype of respiratory diseases.

Paquin-Proulx, DominicKarolinska Univ Hosp Huddinge, Karolinska Inst, Ctr Infect Med, Dept Med, F59, S-14186 Stockholm, Sweden;George Washington Univ, Dept Microbiol Immunol & Trop Med, Washington, DC USA (författare)
Moll, MarkusKarolinska Institutet (författare)
Flodström-Tullberg, MalinKarolinska Univ Hosp Huddinge, Karolinska Inst, Ctr Infect Med, Dept Med, F59, S-14186 Stockholm, Sweden (författare)
Hjelte, LenaKarolinska Institutet (författare)
Sandberg, Johan K.Karolinska Institutet (författare)
Uppsala universitetLung- allergi- och sömnforskning (creator_code:org_t)

Av författaren/redakt...: Pincikova, Terez ...; Paquin-Proulx, D ...; Moll, Markus; Flodström-Tullbe ...; Hjelte, Lena; Sandberg, Johan ...

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