Results 1 to 10 of about 42,619 (218)

Signaling pathways, microenvironment, and targeted treatments in Langerhans cell histiocytosis

Cell Communication and Signaling, 2022
Langerhans cell histiocytosis (LCH) is an inflammatory myeloid malignancy in the “L-group” histiocytosis. Mitogen-activated protein kinase (MAPK) pathway activating mutations are detectable in nearly all LCH lesions. However, the pathogenic roles of MAPK
Xue-min Gao, Jian Li, Xin-xin Cao
doaj   +2 more sources

Adult-onset perianal Langerhans cell histiocytosis presenting as pruritus ani: a case report and review of the literature

Journal of Medical Case Reports, 2021
Background Langerhans cells belong to the histiocytic system and give rise to two tumors: Langerhans cell histiocytosis and Langerhans cell sarcoma. Clinical aggressiveness and degree of atypia distinguish the two neoplasms. Langerhans cell histiocytosis
Marah Hamdan, Jesse C. Qiao, Vid Fikfak
doaj   +2 more sources

Pulmonary Langerhans Cell Histiocytosis Presenting as Multiple Obstructing Bronchial Lesions and Pulmonary Masses [PDF]

The Clinical Respiratory Journal
Pulmonary Langerhans cell histiocytosis is a rare lung disease, which was previously classified as interstitial lung disease but is now classified as a myeloid neoplasm.
Jingyuan Fan, Chen Liu, Zhen Yang, Danxiong Sun   +3 more
doaj   +2 more sources

Efficacy of MAPK inhibitors in children with neurodegenerative Langerhans cell histiocytosis: Results from the European Consortium for Histiocytosis. [PDF]

Hemasphere
Abstract Neurodegenerative Langerhans cell histiocytosis (ND‐LCH) is a severe and debilitating complication of LCH with no effective treatment. In this study, we assessed the efficacy of MAPK inhibitors (MAPKi) in a large cohort of children with ND‐LCH. We included pediatric patients with ND‐LCH who received MAPKi between 2013 and 2025.
Pegoraro F, Chalard F, Evseev D, Barkaoui M, Le Louet S, Thalhammer J, Aladjidi N, Salmon A, Marec-Berard P, Gaspari S, De Fusco C, Trambusti I, Verrico A, Raciborska A, Svojgr K, Křenová Z, Slater O, Millen G, Ben Arush M, Ashkenazi Lustig D, Von Bahr Greenwood T, Hutter C, Astigarraga I, López-Duarte M, Valero-Arrese L, Ortega Acosta MJ, van den Bos C, Naeije L, Martin-Munoz D, Perrone A, Mortilla M, Felician O, Idbaih A, Héritier S, Nanduri V, Minkov M, Henter JI, Sieni E, Donadieu J.   +38 more
europepmc   +2 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

Langerhans cell histiocytosis in adults: Advances in pathophysiology and treatment

Cancer Science, 2018
Langerhans cell histiocytosis (LCH) is a rare systemic disorder characterized by the accumulation of CD1a+/Langerin+ LCH cells and wide‐ranging organ involvement.
Masayuki Kobayashi, Arinobu Tojo
exaly   +2 more sources

RAF/MEK/extracellular signal–related kinase pathway suppresses dendritic cell migration and traps dendritic cells in Langerhans cell histiocytosis lesions

Journal of Experimental Medicine, 2018
Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia characterized by granulomatous lesions containing pathological CD207+ dendritic cells (DCs) with constitutively activated mitogen-activated protein kinase (MAPK) pathway signaling ...
Marie-Luise Berres, Rikhia Chakraborty, Jun Tang   +2 more
exaly   +2 more sources

International expert consensus recommendations for the diagnosis and treatment of Langerhans cell histiocytosis in adults

Blood, 2022
Langerhans cell histiocytosis (LCH) can affect children and adults with a wide variety of clinical manifestations, including unifocal, single-system multifocal, single-system pulmonary (smoking-associated), or multisystem disease.
G. Goyal, A. Tazi, R. Go, K. Rech, J. Picarsic, R. Vassallo, Jason R. Young, C. Cox, J. V. van Laar, M. Hermiston, Xin-xin Cao, P. Makras, G. Kaltsas, J. Haroche, M. Collin, K. McClain, E. Diamond, M. Girschikofsky   +17 more
semanticscholar   +1 more source

Establishment of MOS-SF36 percentile ranks in the general youth French population

BMC Psychology, 2022
Background The SF-36 is a generic quality of life questionnaire, massively translated and widely used to obtain physical and mental health status. However, validation work in the French language was carried out over a generation ago.
Arthur Trognon, Emilie Tinti, Blandine Beaupain, Jean Donadieu, Michel Musiol   +4 more
doaj   +1 more source

Dabrafenib and trametinib in Langerhans cell histiocytosis and other histiocytic disorders

Haematologica, 2023
The standard treatment for Langerhans cell histiocytosis (LCH) is chemotherapy, although the failure rates are high. Since MAP-kinase activating mutations are found in most cases, BRAF- and MEK-inhibitors have been used successfully to treat patients ...
E. Cournoyer, Justin Ferrell, Susan F. Sharp, Anish Ray, Michael Jordan, C. Dandoy, M. Grimley, Somak Roy, Robert Lorsbach, A. C. Merrow, A. Nelson, Allison L. Bartlett, J. Picarsic, Ashish R Kumar   +13 more
semanticscholar   +1 more source