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Immunol Cell Biol. 2019 Jan 11. doi: 10.1111/imcb.12230. [Epub ahead of print]

Widespread alterations of the peripheral blood innate immune cell profile in cystic fibrosis reflect lung pathology and may inform patient management.

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School of Medicine, University of Tasmania, Hobart, TAS, Australia.
Royal Hobart Hospital, Hobart, TAS, Australia.
Centenary Institute, Newtown, NSW, Australia.
Discipline of Pathology, Faculty of Medicine and Health and the Ramaciotti Facility for Human Systems Biology, Charles Perkins Centre, University of Sydney, Sydney, NSW, Australia.


Cystic fibrosis (CF) is caused by mutations to the CF transmembrane conductance regulator (CFTR) gene. The CFTR is known to be expressed on multiple immune cell subtypes, dendritic cells (DCs), monocytes/macrophages, neutrophils and lymphocytes. We hypothesised that lack of CFTR expression on peripheral blood innate immune cells would result in an altered cell profile in the periphery and that this profile would reflect lung pathology. We performed a flow cytometric phenotypic investigation of innate immune cell proportions in peripheral blood collected from 17 CF patients and 15 age-matched healthy controls. We observed significant differences between CF patients and controls in the relative proportions of NK cells, monocytes and their subsets, with significant correlations observed between proportions of NK and monocyte cell subsets and lung function (FEV1% predicted) in CF patients. This study demonstrates the widespread nature of immune dysregulation in CF and provides a basis for identification of potential therapeutic targets. Modulation of the distinct CF-related immune cell phenotype identified could also be an important biomarker for evaluating CFTR-targeted drug efficacy. This article is protected by copyright. All rights reserved.


CD14; CD56; dendritic cells; monocytes; myeloid derived suppressor cells; natural killer cells; peripheral blood; xfibrosis


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