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Erdheim-Chester disease [PDF]

European Journal of Internal Medicine, 2015
Erdheim-Chester disease (ECD) is rare form of non-Langerhans cells histiocytosis with multiorgan involvement. Individuals are more frequently affected in their fifth decade and there is a slight male prevalence. Recent studies have demonstrated that ECD patients bare mutations in the proto-oncogene BRAF (and more rarely in other genes involved in the ...
Corrado Campochiaro, Giulio Cavalli, Alessandro Tomelleri   +2 more
exaly   +14 more sources

Erdheim-Chester disease [PDF]

Indian Journal of Rheumatology, 2013
Erdheim-Chester disease (ECD) is a rare form of non-Langerhans' cell histiocytosis. Diagnosis of ECD is based on the identification in tissue biopsy of histiocytes, which are typically foamy and immunostain for CD68+ CD1a-. Central nervous system involvement is a major prognostic factor in ECD.
Vibhor Wadhwa, Kyung Jin Suh, Avneesh Chhabra   +2 more
doaj   +9 more sources

Erdheim-Chester Disease

JACC: Case Reports, 2020
We describe the case of a 79-year-old woman with a history of Erdheim-Chester disease who presented with bradyarrhythmia and infiltration of the superior vena cava and right atrium.
Michael Tao, MD, Brittany Palmer, MD, Sina Tavakoli, MD, PhD, Humberto E. Trejo Bittar, MD   +3 more
doaj   +11 more sources

ERDHEIM-Chester Disease: Two Fatal Cases With Unusual Presentations. [PDF]

Clin Case Rep
Fluorodeoxyglucose positron emission tomography‐computed tomography (FDG PET‐CT) demonstrated markedly increased metabolic activity in serosal surfaces including pericardium, pleura, and renal fascia, as well as diffuse uptake along vascular walls, particularly the entire aorta.
Daniel Andres RV, Álvarez Payares José C, Juliana MP.   +2 more
europepmc   +2 more sources

Glomeruloid haemangioma in Erdheim-Chester disease: An atypical skin manifestation associated with elevated vascular endothelial growth factor-A levels. [PDF]

Br J Haematol
Glomeruloid haemangiomas and extensive angiomas occurred in a small subset of Erdheim–Chester disease patients, all showing markedly elevated vascular endothelial growth factor‐A (VEGF‐A) levels despite the absence of POEMS (polyneuropathy, organomegaly, M‐spike, and skin disease) syndrome.
Razanamahery J, Papo M, Pegoraro F, Blaison F, Legendre P, Carado V, Caretti M, Helene ALM, Emile JF, Vaglio A, Haroche J.   +10 more
europepmc   +2 more sources

Novel BRAF fusion in Erdheim-Chester disease with pulmonary manifestations: Importance of RNA-based testing and response to MEK inhibition. [PDF]

Histopathology
Histopathology, Volume 89, Issue 3, Page 558-561, September 2026.
Odintsov I, Tazelaar HD, Lee JC, Damon LE, Devine WP, Jones KD.   +5 more
europepmc   +2 more sources

Erdheim–Chester disease mimicking Wilms tumor in a child: a diagnostic challenge [PDF]

BMC Pediatrics
Background Erdheim–Chester disease (ECD) is a rare non-Langerhans cell histiocytosis driven by activating mutations of the MAPK signaling pathway, most commonly BRAF V600E. Pediatric cases are exceedingly rare and often present with atypical, multisystem
Svitozar Khalak, Andrii Nakonechnyi
doaj   +2 more sources

Erdheim-Chester disease associated with myeloid neoplasm: Clinical features, molecular landscape, and treatment outcomes. [PDF]

Hemasphere
Abstract Erdheim‐Chester disease (ECD) is frequently associated with clonal hematopoiesis and myeloid neoplasms (MN), but clinical phenotype and response to kinase inhibitors (KI) in this setting remain unclear. We analyzed 67 patients with ECD associated with MN (ECD‐MN) from a French national cohort and assessed ECD treatment response, MN progression,
Le Pogam A, Papo M, Hirsch P, Cohen-Aubart F, Emile JF, Delhommeau F, Duffau P, Razanamahery J, Nguyen-Khac F, Nunes Gomes C, Triboulet F, de La Rochefoucauld J, McAvoy C, Lazaro E, Hage-Sleiman M, Roos-Weil D, Chasset F, Jachiet V, Mekinian A, Fain O, Amoura Z, Hadjadj J, Haroche J.   +22 more
europepmc   +2 more sources

Erdheim-Chester disease [PDF]

JACC: Case Reports
Erdheim Chester is a rare histiocytic infiltrative disease characterized with bilateral symmetrical sclerotic involvement of the metaphyses and diaphyses of long bones. Fifty-one years old male patient presented with dull knee and leg pain which was present for two years and did not respond to non-steroidal anti-inflammatory medication was evaluated ...
Altinel, Levent, Degirmenci, Bumin, Kose, Kamil Cagri, Sahin, Onder, Ergan, Volkan   +4 more
europepmc   +5 more sources

Multimodality Imaging in a Patient with Erdheim Chester Disease with Cardiovascular Involvement [PDF]

Acta Medica Lituanica
We hereby report a 40-year-old woman presenting with chest pain and recurrent pericardial effusion. Cardiac MRI revealed diffuse sheath-like mass involving predominantly the right heart chambers. She also had bony and perirenal involvement. Additionally,
Priya Jagia, Vineeta Ojha, Gautam Sharma
doaj   +2 more sources