title: Langerhans cell histiocytosis in Chinese adults: absence of BRAF mutations and increased FOXP3(+) regulatory T cells.
abstract: Langerhans cell histiocytosis (LCH) is a rare disorder characterized by the proliferation of abnormal Langerhans cells. Previous studies mainly focused on children with LCH. However, there is limited information on the clinical and pathological aspects of LCH in adults. Therefore, this study aimed to investigate the clinical and pathological aspects of LCH in Chinese adults. The results showed that the average age of 18 LCH patients was 35.22 ± 16.57 years old. The ratio of male to female was 3.5:1.14 patients (77.8%) had single-system involvement and 4 patients (22.2%) had multi-system diseases. The skin (38.9%) and lungs (44.4%) were the mainly affected organs. No BRAF mutations were detected in the lesions of 18 cases. The number of FOXP3(+) Tregs was significantly increased in LCH. In conclusion, clinical features of LCH in adults are distinct from those in children. Adult LCH has a relatively good prognosis and presents as a benign disease. Immune regulation plays an important role in the pathogenesis of adult LCH.
authors.affiliation: Department of Dermatology, Beijing Chao-Yang Hospital, Capital Medical University Beijing 100020, China.
authors.forename: Chunguang
authors.initials: C
authors.lastname: Tong
authors.affiliation: Medical Research Center, Beijing Chao-Yang Hospital, Capital Medical University Beijing 100020, China.
authors.forename: Xingyuan
authors.initials: X
authors.lastname: Jia
authors.affiliation: Medical Research Center, Beijing Chao-Yang Hospital, Capital Medical University Beijing 100020, China.
authors.forename: Yanjun
authors.initials: Y
authors.lastname: Jia
authors.affiliation: Department of Dermatology, Beijing Chao-Yang Hospital, Capital Medical University Beijing 100020, China.
authors.forename: Yanling
authors.initials: Y
authors.lastname: He
mesh_terms: D000328:Adult; D044466:Asian People; D005260:Female; D051858:Forkhead Transcription Factors / Q000276:immunology; D006646:Histiocytosis, Langerhans-Cell / Q000235:genetics* / Q000276:immunology* / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D009154:Mutation; D048493:Proto-Oncogene Proteins B-raf / Q000235:genetics*; D020133:Reverse Transcriptase Polymerase Chain Reaction; D050378:T-Lymphocytes, Regulatory / Q000276:immunology*
chemical_list: C418974:FOXP3 protein, human; D051858:Forkhead Transcription Factors; C482119:BRAF protein, human; D048493:Proto-Oncogene Proteins B-raf
keywords: BRAF; FOXP3+ regulatory T cells; Langerhans cell histiocytosis; mutations