Low periostin levels in adult patients with Langerhans cell histiocytosis are independently associated with the disease activity.

Purpose: Langerhans cell histiocytosis (LCH) is a rare proliferative disease of cells of the CD1a+/CD207+ myeloid dendritic cell lineage that may infiltrate one or more organs or systems at all ages. We aimed to evaluate periostin and sclerostin serum levels in adult patients with LCH.

Procedures: This was a cross-sectional study comparing 38 adult patients with LCH with 38 age- and sex-matched healthy controls. Serum periostin and sclerostin levels were measured to compare between LCH patients and controls as well as between patients with active and non-active disease.

Results: Serum periostin levels were significantly lower in LCH patients than controls (457±72ng/ml vs. 721±79ng/ml, p=0.014) but this was not the case for sclerostin levels which did not differ between patients and controls, respectively (29.0±1.8pmol/L vs. 39.5±3.8pmol/L, p=0.12). Patients with active disease had significantly lower periostin levels than those with inactive disease (240±78ng/ml vs. 558±94ng/ml, p=0.008). No effect of specific site involvement, extend of disease, or treatment administered was found on any of the above parameters measured.

Conclusions: Lower serum periostin levels were observed in adult LCH patients with active disease. The finding warrants further investigation to define whether periostin could serve as a serum biomarker for LCH activity.