IL-31, predominantly produced by CD45RO + CLA + Th2 cells, plays an important pathogenetic role in pruritic
skin diseases like
atopic dermatitis. As
tumor cells in Sézary syndrome (SS) and
Mycosis fungoides (MF) possess similar immunophenotypes and the conditions mentioned are often associated with
pruritus, the analysis of the IL-31 pathway in MF/SS patients is of interest. Serum samples from the peripheral blood of 23 patients and 17 controls were analyzed for IL-31 abundance and correlated with disease stage and
pruritus. Furthermore IL-31-, IL-31 receptor alpha (IL-31Rα)- and
Oncostatin M receptor beta (OSMRβ)-
mRNA expression was measured in blood
tumor cells from SS patients, memory T-cells from controls and
lymphoma cell lines. Serum IL-31 levels were low but differed between groups with no or strong
pruritus. Expression of IL-31 was detectable at low levels in cell lines, but not in the
tumor cells of SS patients. Stimulation with PMA/
ionomycin led to indiscriminate expression in peripheral blood
tumor cells and control T-cells. IL-2-stimulation resulted in expression only in 9/11 patient samples. IL-31Rα-expression was detectable in 10/10 cell lines, 8/15 peripheral blood samples from SS patients, and 4/10 controls; whereas, OSMRβ
mRNA was detectable in 4/10 cell lines, but only one patient and control sample. The results of our analyses regarding serum levels and receptor expression do not suggest a central role of IL-31 in MF/SS pathogenesis. However, the results of
IL-2 stimulation as well as the increased IL-31 levels in patients with strong
pruritus offer a rationale for therapeutic approach in this subset of patients.