A new clinical score for disease activity in Langerhans cell histiocytosis

Objective. To develop an objective tool for assessing disease activity in patients with Langerhans cell histiocytosis (LCH). Method. Scoring system was developed and applied to a database containing information on 612 patients. Results. At diagnosis, the score distribution was highly asymmetrical: the score was between 0 and 2 in 74% of cases, 3-6 in 16%, 7-10 in 39%, and more than 10 in 6%. The 5-year mortality rates were 1, 4.4, and 43.4%, respectively, among patients with initial scores of 0-2, 3-6, and >6. Stability or an increase of the score at 6 weeks was highly predictive of death among patients with initial scores above 6, while score stability had no significant impact on vital outcome among patients with low or moderate scores at diagnosis. Conclusions. This LCH disease activity score provides an objective tool for assessing disease severity, both at diagnosis and during follow-up and treatment. (C) 2004 Wiley-Liss, Inc.