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

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

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

AMER SOC HEMATOLOGY

Año: 2017

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 TSLP and TGFβ plasma levels were measured in 22 pediatric patients with active disease (AD) or non-active disease (NAD). The myeloid compartment showed an increased CD11b (CD11bhigh plus CD11b+) fraction (39.7 ± 3.6 vs 18.6 ± 1.9), a higher percentage of circulating CD11bhighCD11c+CD207+ cells (44.5 ± 11.3 vs 3.2 ± 0.5), and the presence of CD11chighCD207+CD1a+ cells (25.0 ± 9.1 vs 2.3 ± 0.5) in patients with AD vs NAD. Blood CD207+CD1a+ cells were not observed in adult controls or umbilical cord. Increased TSLP and TGFβ levels were detected in patients with AD. Interestingly, plasma from patients with AD induces CD207 expression on CD14+ monocytes. We can conclude that CD207+CD1a+ cells are circulating in patients with active LCH, and TSLP and TGFβ are potential drivers of these LC-like cells in vivo.