Abdominal Extrarenal Involvement in Erdheim-Chester Disease in a Cohort of 304 Patients.
Sign Up to like & getrecommendations! Published in 2022 at "JAMA oncology"
DOI: 10.1001/jamaoncol.2022.4536
Abstract: This cohort study estimates the prevalence of nonretroperitoneal abdominal organ involvement in Erdheim-Chester disease in a large cohort of patients. read more here.
Keywords: involvement erdheim; erdheim chester; cohort; chester disease ... See more keywords
Sustained, complete response to pexidartinib in a patient with CSF1R‐mutated Erdheim–Chester disease
Sign Up to like & getrecommendations! Published in 2022 at "American Journal of Hematology"
DOI: 10.1002/ajh.26441
Abstract: Erdheim–Chester disease (ECD) is a histiocytic neoplasm that predominantly harbors mitogen‐activated protein kinase (MAPK) pathway variants. MAPK inhibitors typically are effective treatments, but mutations outside the MAPK pathway, such as CSF1R variants, may cause refractory… read more here.
Keywords: erdheim chester; csf1r; patient; chester disease ... See more keywords
Adult leukoencephalopathies with prominent infratentorial involvement can be caused by Erdheim–Chester disease
Sign Up to like & getrecommendations! Published in 2017 at "Journal of Neurology"
DOI: 10.1007/s00415-017-8692-8
Abstract: BackgroundLeukoencephalopathies with prominent involvement of cerebellum and brainstem, henceforward called prominent infratentorial leukoencephalopathies (PILs), encompass a variety of inherited and acquired white matter diseases. Erdheim–Chester disease (ECD) is a rare non-Langerhans cell histiocytosis likely under-diagnosed… read more here.
Keywords: erdheim chester; adult; chester disease; disease ... See more keywords
Erdheim-Chester Disease: a Rare but Important Cause of Recurrent Pericarditis
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DOI: 10.1007/s11886-020-01307-z
Abstract: Purpose of Review Erdheim-Chester disease (ECD) is a very rare neoplasm of the non-Langerhans cell histiocytes. Pericardial involvement is uncommon, and we aim to review the current knowledge on the epidemiology, clinical manifestations, and management… read more here.
Keywords: pericarditis; erdheim chester; recurrent pericarditis; chester disease ... See more keywords
Erdheim-Chester disease after Essential Thrombocythemia: Coincidence or not?
Sign Up to like & getrecommendations! Published in 2021 at "Hematology, transfusion and cell therapy"
DOI: 10.1016/j.htct.2021.01.013
Abstract: Erdheim-Chester disease (ECD) is a rare neoplasm from nonLangerhans cell (CD1a -, CD68+, S100-) histiocytes most commonly reported in elderly males. Patients with ECD have been reported to harbor several somatic mutations activating MAPK and… read more here.
Keywords: erdheim chester; essential thrombocythemia; chester disease;
Intracranial Erdheim-Chester Disease Mimicking Parafalcine Meningioma: Report of Two Cases and Review of the Literature.
Sign Up to like & getrecommendations! Published in 2018 at "World neurosurgery"
DOI: 10.1016/j.wneu.2017.11.074
Abstract: BACKGROUND Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis that typically occurs in middle-aged patients. It is usually characterized by multifocal osteosclerotic lesions of the long-bones, however many cases have extraskeletal involvement. Central nervous… read more here.
Keywords: erdheim chester; report two; chester disease; disease ... See more keywords
The histopathology of Erdheim–Chester disease: a comprehensive review of a molecularly characterized cohort
Sign Up to like & getrecommendations! Published in 2017 at "Modern Pathology"
DOI: 10.1038/modpathol.2017.160
Abstract: Erdheim–Chester disease is a rare, non-Langerhans cell histiocytosis histologically characterized by multi-systemic proliferation of mature histiocytes in a background of inflammatory stroma. The disease can involve virtually any organ system; most commonly the bones, skin,… read more here.
Keywords: erdheim chester; chester disease; histopathology; disease ... See more keywords
Erdheim–Chester disease: a rapidly evolving disease model
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DOI: 10.1038/s41375-020-0944-4
Abstract: Erdheim–Chester disease (ECD) is a rare non-Langerhans cell histiocytosis with a putative neoplastic and inflammatory nature. The disease is driven by mutations in proto-oncogenes such as BRAF and MEK , while immune-mediated mechanisms contribute to… read more here.
Keywords: erdheim chester; chester disease; disease; disease rapidly ... See more keywords
Erdheim–Chester disease and vemurafenib: a review of ophthalmic presentations and clinical outcomes
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DOI: 10.1080/01676830.2022.2087232
Abstract: ABSTRACT Purpose To provide a comprehensive review of ocular and orbital manifestations of Erdheim–Chester Disease (ECD) and compare clinical outcomes with vemurafenib (INN) to historical treatments (HT). Primary outcomes are ophthalmic findings on presentation, changes… read more here.
Keywords: clinical outcomes; erdheim chester; review; chester disease ... See more keywords
Clinical, laboratory and genetic features of Erdheim-Chester disease patients: analysis of a retrospective cohort of two reference centers in Latin America.
Sign Up to like & getrecommendations! Published in 2022 at "Hematology"
DOI: 10.1080/16078454.2021.2011547
Abstract: Objectives and Methods: Erdheim-Chester disease (ECD) is a rare histiocytic neoplasm with a heterogeneous clinical course, ranging from localized and asymptomatic bone lesions to a multisystem disease, causing significant morbidity and mortality. There are few… read more here.
Keywords: reference; clinical laboratory; erdheim chester; chester disease ... See more keywords
Prevalence, patterns and outcomes of cardiac involvement in Erdheim-Chester disease.
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DOI: 10.1093/eurheartj/ehac741
Abstract: AIMS Cardiac involvement of Erdheim-Chester disease (ECD), a rare L group histiocytosis, has been reported to be associated with poor outcomes, but systematic studies are lacking. The present study aimed to investigate the prevalence, clinical… read more here.
Keywords: involvement erdheim; erdheim chester; involvement; chester disease ... See more keywords