ArticleHematology. American Society of Hematology. Education Program2020
Histiocytic disorders: insights into novel biology and implications for therapy of Langerhans cell histiocytosis and Erdheim-Chester disease.
Article in Hematology. American Society of Hematology. Education Program, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
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5 citing papers in PubMed, 14 citations in OpenAlex.
- A Kikuchi-Fujimoto syndrome in the pocket of a non-Hodgkin lymphoma: case report with a mini-review of the literature.Annals of medicine and surgery (2012) · 2025Article
- Unraveling cutaneous histiocytosis: insights into histology, pathogenesis, diagnosis, and treatment pitfalls.Frontiers in medicine · 2025Review
- Erdheim-Chester Disease in Adults: 11 Cases from a Canadian Rare Diseases Program.Clinical hematology international · 2025Article
- A Rare Case of Erdheim-Chester Disease with Pseudoprogression in the CNS.Journal of blood medicine · 2025Article
- Article
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Abstract
Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD) are caused by mutations of the MAPK pathway, most often BRAFV600E, in myeloid dendritic cells that lead to some overlapping and other unique presentations of the two diseases. LCH occurs in both children and adults, but ECD is primarily found in the latter. The challenges in diagnosing these conditions relates to the rarity of the conditions and that they mimic diseases that are more widely understood, such as certain rashes; bone, lung, and renal diseases; and other malignancies. The histopathology of LCH is definitive, but not so for ECD. Treatment with BRAF and MEK inhibitors has become one of the important advances in the care of these patients.
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