ArticleEClinicalMedicine2024
Long-term outcome and prognosis of mixed histiocytosis (Erdheim-Chester disease and Langerhans Cell Histiocytosis).
Article in EClinicalMedicine, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed.
- Article
- Histiocytosis development and clinical variation through the lens of genomics.The Journal of pathology · 2026Review
- Literature review of pediatric Erdheim-Chester disease (ECD) and mixed histiocytosis (Langerhans cell histiocytosis/ECD).Annals of translational medicine · 2026Review
- A case of mixed histiocytosis (Erdheim-Chester disease and Langerhans cell histiocytosis) with STEAP3-associated anemia and type 4 hemochromatosis.Orphanet journal of rare diseases · 2026Article
- Erdheim-Chester disease associated with myeloid neoplasm: Clinical features, molecular landscape, and treatment outcomes.HemaSphere · 2026Article
- Histiocytoses and reactive proliferations of histiocytes: current state of the art and evolving concepts-a report from the joint CSHP-EA4HP-SH workshop 2024, Hefei, China.Virchows Archiv : an international journal of pathology · 2026Review
- Delayed Neurologic Response to Dabrafenib and Trametinib in the Case of Mixed Histiocytosis (LCH/ECD): Case Report and Literature Review.Reports (MDPI) · 2026Article
- The Challenge of Differentiating Mixed Histiocytosis: A Case Report.Case reports in hematology · 2026Article
- When Erdheim-Chester Disease Reaches the Pectoral Muscle.European journal of case reports in internal medicine · 2026Article
- Mixed Erdheim-Chester disease with thoraco-abdominal involvement.Acta radiologica open · 2025Article
- Histiocyte Society blueprint for Langerhans cell histiocytosis research: from cell-of-origin to a more comprehensive cure.Haematologica · 2025Review
- Application of pretreatmentAnnals of hematology · 2025Article
- Efficacy and safety of Dabrafenib in Erdheim-Chester disease.Annals of hematology · 2025Article
- Article
- MEK inhibitors are effective in Erdheim-Chester disease regardless of MAPK pathway mutations.Leukemia · 2025Article
- Treatment holidays in patients with Erdheim-Chester disease receiving vemurafenib: a prospective pilot study.Blood advances · 2025Article
- Risk of Second Cancer in Erdheim-Chester Disease.JAMA oncology · 2025Article
- Overlap syndrome of Erdheim-Chester disease and Langerhans cell histiocytosis: A case report.CytoJournal · 2024Article
Corrections and comments
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Authors and funding
37 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
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 Interpretation: Mixed ECD-LCH is a multisystem disease driven by the Funding: None.
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