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

Eugenio Antonio Carrera Silva, Wanda Nowak, Licina Tessone, Cinthia Mariel Olexen, Juan Manuel Ortiz Wilczyñski, Ivana Gisele Estecho, Graciela Elena, Andrea Emilse Errasti and Diego Alfredo Rosso

Key points

  • CD207+CD1a+ cells are present in the circulation of patients with active LCH.

  • TGFβ and TSLP are increased in the plasma of patients with active LCH.


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.

  • Submitted May 3, 2017.
  • Accepted August 16, 2017.