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

TESSONE, LICINA; ESTECHO, IVANA GISELE; ROSSO, DIEGO ALFREDO; TESSONE, LICINA; SILVA, EUGENIO ANTONIO CARRERA; ESTECHO, IVANA GISELE; OLEXEN, CINTHIA MARIEL; ROSSO, DIEGO ALFREDO; ELENA, GRACIELA; SILVA, EUGENIO ANTONIO CARRERA; OLEXEN, CINTHIA MARIEL; ELENA, GRACIELA; NOWAK, WANDA; WILCZYNSKI, JUAN MANUEL ORTIZ; ERRASTI, ANDREA EMILSE; NOWAK, WANDA; WILCZYNSKI, JUAN MANUEL ORTIZ; ERRASTI, ANDREA EMILSE

BLOOD, THE JOURNAL OF THE AMERICAN SOCIETY OF HEMATOLOGY - PRINT

AMER SOC HEMATOLOGY

Año: 2017 vol. 130 p. 1898 - 1902

0006-4971

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 ADvs those with NAD, themyeloid compartment showed an increased CD11b (CD11bhigh plusCD11b+) fraction (39.763. ± vs 18. ±61.9), a higher percentage of circulating CD11bhighCD11c1CD2071 cells (44.5±11.3 vs 3.2±0.5), and the presence of CD11chigh 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-b 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-b are potential drivers of Langerhans-like cells in vivo.