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dc.contributor.authorAstigarraga Aguirre, María Iciar
dc.contributor.authorGarcía Obregón, Susana
dc.contributor.authorPérez Martínez, Antonio
dc.contributor.authorGutiérrez Carrasco, Ignacio
dc.contributor.authorSanta-María López, Vicente
dc.contributor.authorRodríguez-Vigil Iturrate, Carmen
dc.contributor.authorLorite Reggiori, Mikael
dc.contributor.authorMurciano Carrillo, Thais
dc.contributor.authorTorrent, Montse
dc.contributor.authorGrupo de Histiocitosis de la Sociedad Española de Hematología y Oncología Pediátricas (SEHOP)
dc.date.accessioned2022-09-21T16:16:20Z
dc.date.available2022-09-21T16:16:20Z
dc.date.issued2022-08
dc.identifier.citationAnales de Pediatría (English Edition) 97(2) : 130.e1-130.e7 (2022)es_ES
dc.identifier.issn2341-2879
dc.identifier.urihttp://hdl.handle.net/10810/57802
dc.description.abstractLangerhans cell histiocytosis (LCH) is a type of myeloid neoplasia that can affect different organs or tissues and exhibits substantial variability in its clinical presentation and biological behaviour, so it may mimic different diseases. Performance of different clinical assessments and laboratory and imaging tests is recommended to determine the extent of involvement, which may be of a single location or multisystemic, and the presence or absence of dysfunction in risk organs, such as the haematopoietic system, liver and spleen. The diagnosis must be confirmed by histological examination of a biopsy sample. Molecular tests have identified mutations in the mitogen-activated protein kinase (MAPK) pathway, which has expanded treatment options. The diagnosis is complex and there is controversy regarding the management of certain cases. Treatment recommendations depend on the location of the lesions and the extent of involvement. International collaborative studies have demonstrated the effectiveness of prolonged combination therapies such as vinblastine and prednisone in severe or multisystemic forms, and anti-inflammatory drugs such as indomethacin and other cytostatic combinations have proven beneficial. Langerhans cell histiocytosis is a good example of the importance of precision medicine and the benefit of identifying molecular targets, common to different neoplasms, to develop new therapies. MAPK pathway inhibitors offer an alternative treatment option in refractory cases and neurodegenerative forms of LCH. Molecular testing can contribute to the prognosis, treatment and follow-up of LCH, especially in severe forms of disease.es_ES
dc.description.sponsorshipThe study was partially funded by a grant allocated to the Histiocytosis Research Project of the Fundación Vasca de Innovación e Investigación Sanitaria BIOEF (BIO16/ER/020/BC) and the Asociación Española contra la Histiocitosis-ACHE (BC/A/15/012, BIOEF11/017, BIOEF09/047), whose principal investigator is Itziar Astigarraga.es_ES
dc.language.isoenges_ES
dc.publisherElsevieres_ES
dc.rightsinfo:eu-repo/semantics/openAccesses_ES
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/3.0/es/*
dc.subjecthistiocytosises_ES
dc.subjecthistiocytic disorderses_ES
dc.subjectlangerhans cell histiocytosises_ES
dc.subjecteosinophilic granulomaes_ES
dc.subjecttargeted therapyes_ES
dc.subjectBRAF inhibitorses_ES
dc.subjectMEK inhibitorses_ES
dc.subjectprecision medicinees_ES
dc.titleLangerhans cell histiocytosis. Advances in pathogenesis and clinical practice.es_ES
dc.typeinfo:eu-repo/semantics/articlees_ES
dc.rights.holder© 2022 Asociaci ́on Espa ̃nola de Pediatr ́ıa. Published by Elsevier Espa ̃na, S.L.U. This is an open access article under the CC BY-NC-NDlicense (http://creativecommons.org/licenses/by-nc-nd/4.0/)es_ES
dc.rights.holderAtribución-NoComercial-SinDerivadas 3.0 España*
dc.relation.publisherversionhttps://www.sciencedirect.com/science/article/pii/S234128792200148X?via%3Dihubes_ES
dc.identifier.doi10.1016/j.anpede.2022.05.005
dc.departamentoesPediatríaes_ES
dc.departamentoeuPediatriaes_ES


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© 2022 Asociaci ́on Espa ̃nola de Pediatr ́ıa. Published by Elsevier Espa ̃na, S.L.U. This is an open access article under the CC BY-NC-NDlicense (http://creativecommons.org/licenses/by-nc-nd/4.0/)
Except where otherwise noted, this item's license is described as © 2022 Asociaci ́on Espa ̃nola de Pediatr ́ıa. Published by Elsevier Espa ̃na, S.L.U. This is an open access article under the CC BY-NC-NDlicense (http://creativecommons.org/licenses/by-nc-nd/4.0/)