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Incidence of Langerhans cell histiocytosis in children: A population‐based study
Author(s) -
Stålemark Helen,
Laurencikas Evaldas,
Karis Jenny,
Gavhed Désirée,
Fadeel Bengt,
Henter JanInge
Publication year - 2008
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.21504
Subject(s) - medicine , langerhans cell histiocytosis , incidence (geometry) , histiocytosis , pediatrics , population , dermatology , pathology , disease , environmental health , physics , optics
Background Langerhans cell histiocytosis is a rare disease of unknown etiology. We wanted to assess the population‐based incidence of LCH in a well‐defined cohort of children. Methods We identified all children <15‐years old treated with LCH during the 10 years period 1992–2001 at the Department of Pediatrics, Karolinska University Hospital in Stockholm, the referral center for children with LCH in Stockholm County. We also contacted the Departments of Dermatology, Orthopedics, and Neurosurgery for possible additional patients. Results Twenty‐nine children (16 males) with LCH were identified, with a median age at diagnosis of 3.8 years (2 months–13.7 years). All children but one had a definitive diagnosis of LCH. The minimum incidence of LCH is estimated to 8.9/10 6 children per year. At diagnosis, 20 children (69%) had single system (SS) and 9 (31%) multisystem (MS) manifestations. Five of the 20 children with SS eventually developed MS disease, thus 14 (48%) had MS involvement at the maximal extent of disease (4.3/10 6 children per year). Interestingly, 22 children (76%) were diagnosed during the fall (September–November, n = 12) and winter (December–February, n = 10) seasons, as compared to seven children during the spring (March–May = 1) and summer (June–August = 6) seasons ( P = 0.005, Chi‐square). Conclusions The incidence of childhood LCH in our study is higher than previously reported. In our patient cohort, LCH was more commonly diagnosed during the fall and winter season as compared to the spring and summer season. Whether this seasonal variation can be confirmed in larger studies and whether it has relevance for LCH pathophysiology remains to be elucidated. Pediatr Blood Cancer 2008;51:76–81. © 2008 Wiley‐Liss, Inc.