Incidence of Langerhans cell histiocytosis in children: A population‐based study

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Abstract
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 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 (4.3/106 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.