Results 21 to 30 of about 4,675 (181)

Erdheim-Chester Disease and Small Lymphocytic Lymphoma Collision Tumour Presenting as a Perirenal Mass

Case Reports in Pathology, 2020
Background. Erdheim-Chester disease is a rare histiocytic neoplasm associated with MAPK pathway mutations. Disease manifestation is variable often involving many different organs, mainly bone, retroperitoneum, the heart, and the central nervous system ...
Antonio Maietta, Maryam Razmpoosh
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 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

Pericardiectomy and Mechanical Mitro-Aortic Valve Replacement in a Young Patient With Erdheim-Chester Disease [PDF]

Texas Heart Institute Journal
Erdheim-Chester disease is a rare histiocytosis that primarily affects the skeletal system, but cardiovascular manifestations occur in 75% of cases and are associated with a poor prognosis. Given the small number of cases, the evolution and management of
Nerea Lopez Perez, MBBS, Mathieu van Steenberghe, MD, PhD, Jörg D. Seebach, MD, Philippe Meyer, MD, Johannes Alexander Lobrinus, MD, Christoph Huber, MD, Mustafa Cikirikcioglu, MD, PhD   +6 more
doaj   +2 more sources

Clinical Images: Erdheim-Chester disease presenting with fever, enlarged lymph nodes, and monoallelic BRAF (V600E) mutation. [PDF]

ACR Open Rheumatol
ACR Open Rheumatology, Volume 8, Issue 3, March 2026.
Gan L, Zhang Q, Yan H, Bai H, Tang X.
europepmc   +2 more sources

Erdheim-Chester disease [PDF]

Journal of the Royal Society of Medicine, 2005
Dr Lee and his colleagues (April 2005 JRSM1) describe a case of renal failure in which the radiographic appearance of bone pointed to Erdheim–Chester disease, a histiocytosis. Among the organs affected in this condition they mention pericardium. We think it important to emphasize the frequency with which heart, pericardium and great vessels are ...
Rhodes, B, Jawad, A S M
openaire   +3 more sources

Erdheim-Chester disease, moving away from the orphan diseases: A case report

Respiratory Medicine Case Reports, 2017
With approximately 750 cases reported, Erdheim-Chester disease is an exceedingly rare histiocyte cell disorder. Affected sites typically include long bones, large vessels and central nervous system.
Jessica M. Stempel, Jean G. Bustamante Alvarez, Andres Mora Carpio, Varun Mittal, Claudia Dourado   +4 more
doaj   +2 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

Case of Erdheim–Chester presenting with xanthelasma-like eruption and osteolytic bone lesions: A case report

SAGE Open Medical Case Reports, 2019
Erdheim–Chester disease is a rare multisystemic non-Langerhans cell histiocytosis presenting 95% with skeletal lesions. Erdheim–Chester disease is due to mutations in the RAS-MEK-ERK pathway where 50% are due to BRAF-V600E mutations.
Evelyn Alarcon Chinchilla, Marie-Pascale Gourde, Karine Turcotte, Steve Mathieu, Mohamed Amin-Hashem   +4 more
doaj   +2 more sources

Updates on Erdheim-Chester disease

Human Pathology Reports, 2022
Erdheim-Chester disease (ECD) is a non-Langerhans cell histiocytosis characterized by multisystemic proliferation of histiocytes in a background of fibrosis and inflammation.
Ismail Elbaz Younes, Austin Ellis, Xiaohui Zhang   +2 more
doaj   +2 more sources