Title of article
Langerhans cell histiocytosis in children – a disease with many faces. Recent advances in pathogenesis, diagnostic examinations and treatment
Author/Authors
Jezierska, Michalina Department of Pediatrics - Hematology and Oncology - Medical University of Gdansk, Poland , Stefanowicz, Joanna Department of Pediatrics - Hematology and Oncology - Medical University of Gdansk, Poland , Romanowicz, Grzegorz Department of Nuclear Medicine - Medical University of Gdansk, Poland , Kosiak, Wojciech Department of Pediatrics - Hematology and Oncology - Medical University of Gdansk, Poland , Lange, Magdalena Department of Dermatology - Venereology and Allergology - Medical University of Gdansk, Poland
Pages
12
From page
6
To page
17
Abstract
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.
Keywords
Langerhans cell histiocytosis , children , pathogenesis , symptoms , treatment
Journal title
Advances in Dermatology and Allergology/Postȩpy Dermatologii i Alergologii
Serial Year
2018
Record number
2623155
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