CD207 + CD1a + cells circulate in pediatric patients with active Langerhans cell histiocytosis.

Autor: Carrera Silva EA; Instituto de Medicina Experimental, Academia Nacional de Medicina, Consejo Nacional de Investigaciones Científicas y Técnicas, Buenos Aires, Argentina., Nowak W; Instituto de Farmacología, Facultad de Medicina, Universidad de Buenos Aires, Buenos Aires, Argentina; and., Tessone L; Instituto de Farmacología, Facultad de Medicina, Universidad de Buenos Aires, Buenos Aires, Argentina; and., Olexen CM; Instituto de Medicina Experimental, Academia Nacional de Medicina, Consejo Nacional de Investigaciones Científicas y Técnicas, Buenos Aires, Argentina.; Instituto de Farmacología, Facultad de Medicina, Universidad de Buenos Aires, Buenos Aires, Argentina; and., Ortiz Wilczyñski JM; Instituto de Medicina Experimental, Academia Nacional de Medicina, Consejo Nacional de Investigaciones Científicas y Técnicas, Buenos Aires, Argentina., Estecho IG; Instituto de Farmacología, Facultad de Medicina, Universidad de Buenos Aires, Buenos Aires, Argentina; and., Elena G; Hospital de Niños Pedro de Elizalde, Buenos Aires, Argentina., Errasti AE; Instituto de Farmacología, Facultad de Medicina, Universidad de Buenos Aires, Buenos Aires, Argentina; and., Rosso DA; Instituto de Farmacología, Facultad de Medicina, Universidad de Buenos Aires, Buenos Aires, Argentina; and.; Hospital de Niños Pedro de Elizalde, Buenos Aires, Argentina.
Jazyk: angličtina
Zdroj: Blood [Blood] 2017 Oct 26; Vol. 130 (17), pp. 1898-1902. Date of Electronic Publication: 2017 Aug 28.
DOI: 10.1182/blood-2017-05-782730
Abstrakt: Langerhans cell histiocytosis (LCH) is a rare disease with an unknown etiology characterized by heterogeneous lesions containing CD207 + CD1a + cells that can arise in almost any tissue and cause significant morbidity and mortality. Precursors of pathological Langerhans cells have yet to be defined. Our aim was to identify circulating CD207 + CD1a + cells and their inducers in LCH. Expression of CD207 and CD1a in the blood myeloid compartment as well as thymic stromal lymphopoietin (TSLP) and transforming growth factor β (TGF-β) plasma levels were measured in 22 pediatric patients with active disease (AD) or nonactive disease (NAD). In patients with AD vs those with NAD, the myeloid compartment showed an increased CD11b (CD11b high plus CD11b + ) fraction (39.7 ± 3.6 vs 18.6 ± 1.9), a higher percentage of circulating CD11b high CD11c + CD207 + cells (44.5 ± 11.3 vs 3.2 ± 0.5), and the presence of CD11c high CD207 + CD1a + cells (25.0 ± 9.1 vs 2.3 ± 0.5). Blood CD207 + CD1a + cells were not observed in adult controls or umbilical cord. Increased TSLP and TGF-β levels were detected in patients with AD. Interestingly, plasma from patients with AD induces CD207 expression on CD14 + monocytes. We conclude that CD207 + CD1a + cells are circulating in patients with active LCH, and TSLP and TGF-β are potential drivers of Langerhans-like cells in vivo.
(© 2017 by The American Society of Hematology.)
Databáze: MEDLINE