Outcome in children with pulmonary Langerhans cell Histiocytosis.

Background The aim of this study was to evaluate features and outcome of children with Langerhans cell Histiocytosis (LCH) and pulmonary involvement. Procedure Retrospective evaluation of LCH patients was performed from 1987 to 2001. Multisystem patients were classified according to the pattern of organ system involvement into Groups: A (no pulmonary, hematologic, or hepatic involvement), B (pulmonary involvement), C (pulmonary and hematologic or hepatic involvement), and D (hematologic or hepatic involvement). All had skin, bone, or lymph node involvement. Chest X-ray was performed in all patients and computed tomography (CT) in 21. Diagnostic lung biopsy was performed in five patients. Results Pulmonary involvement was found in 36/220 patients studied. Two patients had isolated pulmonary involvement. Multisystem involvement was present in 83 patients, 34 of whom had pulmonary involvement. In 20/36 patients, tachypnea, cough, and thoracic pain occurred. Diffuse interstitial involvement was found in all cases. Pulmonary function tests were performed in nine patients, six of whom revealed mild to moderate restrictive respiratory involvement. The two patients having isolated pulmonary involvement survived 2 and 2.7 years after diagnosis. Median follow-up of all multisystem patients was 2.1 years, with a 5-year survival probability of 0.59. The 5-year survival probability of Groups A–D was 0.94, 0.83, 0.23, and 0.40, respectively. The survival difference between Groups B and C was statistically different (P < 0.0071). Conclusions According to our data, pulmonary compromise without other risk organ involvement does not appear to be a negative prognostic factor in our study. © 2004 Wiley-Liss, Inc.