1. Long-term outcome and prognosis of mixed histiocytosis (Erdheim-Chester disease and Langerhans Cell Histiocytosis)Research in context
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Francesco Pegoraro, Matthias Papo, Fleur Cohen-Aubart, Francesco Peyronel, Gianmarco Lugli, Irene Trambusti, Gildas Baulier, Mathilde de Menthon, Tanguy Le Scornet, Eric Oziol, Nicole Ferreira-Maldent, Olivier Hermine, Benoit Faucher, Dirk Koschel, Nicole Straetmans, Noémie Abisror, Benjamin Terrier, François Lifermann, Jerome Razanamahery, Yves Allenbach, Jeremy Keraen, Sophie Bulifon, Baptiste Hervier, Annamaria Buccoliero, Frederic Charlotte, Quentin Monzani, Samia Boussouar, Natalia Shor, Annalisa Tondo, Stephane Barete, Ahmed Idbaih, Abdellatif Tazi, Elena Sieni, Zahir Amoura, Jean-François Emile, Augusto Vaglio, and Julien Haroche
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Erdheim-Chester disease ,Langerhans ,BRAF ,Histiocytosis ,Mixed histiocytosis ,Pediatric histiocytosis ,Medicine (General) ,R5-920 - Abstract
Summary: Background: Erdheim-Chester disease (ECD) is a rare histiocytosis that may overlap with Langerhans Cell Histiocytosis (LCH). This “mixed” entity is poorly characterized. We here investigated the clinical phenotype, outcome, and prognostic factors of a large cohort of patients with mixed ECD-LCH. Methods: This retrospective study was performed at two referral centers in France and Italy (Pitié-Salpêtrière Hospital, Paris; Meyer Children’s Hospital, Florence). We included children and adults with ECD diagnosed in 2000–2022 who had biopsy-proven LCH, available data on clinical presentation, treatment and outcome, and a minimum follow-up of one year. Outcomes included differences in clinical presentation and survival between mixed ECD-LCH and isolated ECD; we also investigated response to treatments and predictors of survival in the mixed cohort. Survival was analyzed using the Kaplan-Maier method and differences in survival with the long-rank test. Cox regression models were used to evaluate the potential impact of age and gender on survival and to identify predictors of non-response and survival. Findings: Out of a cohort of 502 ECD patients, 69 (14%) had mixed ECD-LCH. Compared to isolated ECD, mixed ECD-LCH occurred more frequently in females (51 vs. 26%, p
- Published
- 2024
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