C. Schultz et al., LANGERHANS CELL HISTIOCYTOSIS IN CHILDREN - DOES SOLUBLE INTERLEUKIN-2-RECEPTOR CORRELATE WITH BOTH DISEASE EXTENT AND ACTIVITY, Medical and pediatric oncology, 31(2), 1998, pp. 61-65
Background. Langerhans cell histiocytosis (LCH) is characterized by mo
noclonal proliferation of activated Langerhans cells. Neither etiology
nor pathomechanism oi this disorder is presently known. However, desp
ite monoclonality LCH might represent a reactive clonal disorder induc
ed by immune dysfunction rather than a malignant process. To investiga
te a putative cytokine dysregulation in the pathogenesis of this disor
der and searching for parameters of both disease activity and prognosi
s, serum concentrations of proinflammatory and T-cell derived cytokine
s were evaluated in LCH patients. Materials and Methods. Serum levels
of IL-1 beta, IL-2, sIL-2R and TNF-alpha were determined by ELISA in s
even children with different types of LCH: Three children (aged 6, 10
and 14 years, respectively) with single system/single bone disease; on
e child (11 years) with recurrent single system/multiple bone disease
and three children (1, 2 and 2 years, respectively) with multisystem d
isease. Results. sIL-2R was elevated at diagnosis in seven children as
compared to healthy adults (mean +/- SEM: 5,256 +/-. 3,751 U/ml vs. 7
3 +/- 5.5 U/ml; P<0.005) or healthy children (mean +/- SEM: 10,195 +/-
2,798 pg/ml vs. 2,638 +/- 156 pg/ml; P< 0.01). A positive correlation
between serum levels of sIL-2R and extent of the disease could be obs
erved. During remission, sIL-2R levels declined. IL-l beta, IL-2, and
TNF-alpha remained within the normal range during the study period. Co
nclusions. Elevated sIL-2R levels seem to correlate positively with bo
th extent and activity of LCH, thus indicating a pathological T-cell a
ctivation as a pathogenetic factor. sIL-2R level is a promising parame
ter to monitor disease activity in LCH and may also be of prognostic r
elevance. (C) 1998 Wiley-Liss, Inc.