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Prognostic value of soluble interleukin 2 receptor levels in Langerhans cell histiocytosis
Author(s) -
Rosso Diego A.,
Roy Adriana,
Zelazko Marta,
Braier Jorge L.
Publication year - 2002
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1046/j.1365-2141.2002.03400.x
Subject(s) - gastroenterology , medicine , langerhans cell histiocytosis , histiocyte , histiocytosis , receptor , clinical significance , pathology , immunology , endocrinology , disease
Summary. We investigated the prognostic significance of soluble interleukin 2 receptor (sIL‐2r) levels in the pre‐ and post‐treatment serum of paediatric patients with Langerhans cell histiocytosis (LCH). Serum levels of sIL‐2r from 32 LCH patients and 14 healthy controls were determined using enzyme‐linked immunosorbent assay. The LCH patients were classified, evaluated and treated according to the Histiocyte Society's protocols. The following clinical stages were considered: single‐system disease (A) divided into single‐site (A1; n =4), multiple‐site (A2; n =9), and multisystem disease (B) without organ dysfunction (B1; n =5) and with organ dysfunction (B2; n =14). Pretreatment concentrations of sIL‐2r were markedly increased at diagnosis in LCH patients compared with controls [in pg/ml, median (range) 9200 (1124–40000) versus 610 (343–800)], P < 0·0001. Levels differed significantly between stages A [3250 (1124–11000)] and B [22750 (3400–40000)], P < 0·05, and between substages A2 and B2, P < 0·05. There was a significant correlation between clinical stages and sIL‐2r serum levels, r =0·7996 ( P < 0·0001). Patients with ≥ 17500 pg/ml of sIL‐2r had a 30‐month survival of 0·417 (SEM: 0·142) compared with those with levels < 17500 pg/ml, who presented a 30‐month survival of 0·848 (SEM: 0·100) (log‐rank, P < 0·0001). In multivariate analysis, sIL‐2r levels ≥ 17500 pg/ml were found to have greater predictive strength than other well‐known prognostic factors.