Abstract:
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 3%, 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.
journal_name
Pediatr Blood Cancerjournal_title
Pediatric blood & cancerauthors
Donadieu J,Piguet C,Bernard F,Barkaoui M,Ouache M,Bertrand Y,Ibrahim H,Emile JF,Hermine O,Tazi A,Genereau T,Thomas Cdoi
10.1002/pbc.20160keywords:
subject
Has Abstractpub_date
2004-12-01 00:00:00pages
770-6issue
7eissn
1545-5009issn
1545-5017journal_volume
43pub_type
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