Histiocitosis de Langerhans. Experiencia en edad Pediátrica de 7 años en un hospital de concentración en México.

BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare disease that affects multiple organs and/or systems. There are no descriptions of the experience in Mexican referral hospital OBJECTIVE: To describe the clinical characteristics and response to treatment of patients with LCH. MATERIALS AND METHODS: A descriptive, retrospective and longitudinal study was conducted in a tertiary health care center during 2014 to 2021. Files of pediatric patients with histopathological diagnosis of LCH were reviewed. Descriptive statistics were used. RESULTS: Records of 13 patients were evaluated, 61.5% women, mean age 41.23 months (SD 48.29), and 12(92.3%) with multisystem disease. Of the total, 8(61.5%) required second-line treatment or more. There were 2(14.2%) deaths due to disease progression and 1(7.14%) due to complications of hematopoietic progenitor cell transplantation. CONCLUSIONS: There were 13 cases of LCH in children in 7 years, it is a more frequent disease in children. The mean age of presentation was 41.23 months; predominantly female. Global mortality was 23%, mean follow-up of 34.15 months. High-risk multisystemic disease was the most frequent with 61.5%, mortality of 37.5% and mean follow-up of 32 months. [ABSTRACT FROM AUTHOR]