Langerhans cell histiocytosis in children – a disease with many faces. Recent advances in pathogenesis, diagnostic examinations and treatment

Langerhans cell histiocytosis is a rare clonal disease characterized by the proliferation of CD1a-positive immature dendritic cells. The purpose of this article was to present an updated review of recent advances in the pathogenesis, clinical features, imaging and treatment of this disease. The disc...

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Autores principales: Jezierska, Michalina, Stefanowicz, Joanna, Romanowicz, Grzegorz, Kosiak, Wojciech, Lange, Magdalena
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Termedia Publishing House 2018
Materias:
Review Paper
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5872238/
https://www.ncbi.nlm.nih.gov/pubmed/29599667
http://dx.doi.org/10.5114/pdia.2017.67095
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author Jezierska, Michalina
Stefanowicz, Joanna
Romanowicz, Grzegorz
Kosiak, Wojciech
Lange, Magdalena
author_facet Jezierska, Michalina
Stefanowicz, Joanna
Romanowicz, Grzegorz
Kosiak, Wojciech
Lange, Magdalena
author_sort Jezierska, Michalina
collection PubMed
description Langerhans cell histiocytosis is a rare clonal disease characterized by the proliferation of CD1a-positive immature dendritic cells. The purpose of this article was to present an updated review of recent advances in the pathogenesis, clinical features, imaging and treatment of this disease. The discovery of oncogenic BRAF mutations and the presence of proinflammatory cytokines and chemokines confirmed the unusual characteristics of this disease. Currently, children with organ involvement who do not have a good response to chemotherapy and have neurodegeneration or diabetes insipidus are the most problematic patients. Further research is needed to improve the results of treatment.
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spelling pubmed-58722382018-03-29 Langerhans cell histiocytosis in children – a disease with many faces. Recent advances in pathogenesis, diagnostic examinations and treatment Jezierska, Michalina Stefanowicz, Joanna Romanowicz, Grzegorz Kosiak, Wojciech Lange, Magdalena Postepy Dermatol Alergol Review Paper Langerhans cell histiocytosis is a rare clonal disease characterized by the proliferation of CD1a-positive immature dendritic cells. The purpose of this article was to present an updated review of recent advances in the pathogenesis, clinical features, imaging and treatment of this disease. The discovery of oncogenic BRAF mutations and the presence of proinflammatory cytokines and chemokines confirmed the unusual characteristics of this disease. Currently, children with organ involvement who do not have a good response to chemotherapy and have neurodegeneration or diabetes insipidus are the most problematic patients. Further research is needed to improve the results of treatment. Termedia Publishing House 2018-02-20 2018-02 /pmc/articles/PMC5872238/ /pubmed/29599667 http://dx.doi.org/10.5114/pdia.2017.67095 Text en Copyright: © 2018 Termedia Sp. z o. o. http://creativecommons.org/licenses/by-nc-sa/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0) License, allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
spellingShingle Review Paper
Jezierska, Michalina
Stefanowicz, Joanna
Romanowicz, Grzegorz
Kosiak, Wojciech
Lange, Magdalena
Langerhans cell histiocytosis in children – a disease with many faces. Recent advances in pathogenesis, diagnostic examinations and treatment
title Langerhans cell histiocytosis in children – a disease with many faces. Recent advances in pathogenesis, diagnostic examinations and treatment
title_full Langerhans cell histiocytosis in children – a disease with many faces. Recent advances in pathogenesis, diagnostic examinations and treatment
title_fullStr Langerhans cell histiocytosis in children – a disease with many faces. Recent advances in pathogenesis, diagnostic examinations and treatment
title_full_unstemmed Langerhans cell histiocytosis in children – a disease with many faces. Recent advances in pathogenesis, diagnostic examinations and treatment
title_short Langerhans cell histiocytosis in children – a disease with many faces. Recent advances in pathogenesis, diagnostic examinations and treatment
title_sort langerhans cell histiocytosis in children – a disease with many faces. recent advances in pathogenesis, diagnostic examinations and treatment
topic Review Paper
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5872238/
https://www.ncbi.nlm.nih.gov/pubmed/29599667
http://dx.doi.org/10.5114/pdia.2017.67095
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