LANGERHANS CELL HISTIOCYTOSIS IN CHILDREN - DOES SOLUBLE INTERLEUKIN-2-RECEPTOR CORRELATE WITH BOTH DISEASE EXTENT AND ACTIVITY

Citation
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
Citations number
25
Categorie Soggetti
Oncology,Pediatrics
ISSN journal
00981532
Volume
31
Issue
2
Year of publication
1998
Pages
61 - 65
Database
ISI
SICI code
0098-1532(1998)31:2<61:LCHIC->2.0.ZU;2-A
Abstract
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.