Adult Langerhans cell histiocytosis:A case report and literature review

VernacularTitle:成人郎格汉斯细胞组织细胞增生症1例报告及文献复习
Author: Quan WEN ; Yuanyuan WANG ; Henan SI ; Yaping TIAN
Publication Type:Journal Article
Keywords: langerhans cell histiocytosis; CD1a; diabetes insipidus; immunohistochemistry
From: Journal of Jilin University(Medicine Edition) 2016;42(5):985-987
CountryChina
Language:Chinese
Abstract: Objective:To study the clinical and pathological features of Langerhans cell histiocytosis (LCH), and to provide the reference for its diagnosis and treatment.Methods:The manifestation of one LCH patient was retrospectively analyzed.The features of the LCH patients were explored by analyzing the results of skin biopsy, radiological test and follow-up.The associated literatures were reviewed.Results:The patient presented the typical symptoms gradually,including polycystic lung,skin ulcer,diabetes insipidus,and lactation.The skin pathological findings showed the densely distributed mononuclear cell infiltration in dermis and the immunohistological staining result showed positive CD1a. The patient was follwed up for 7 years and died of heart and lung failure. Conclusion:LHC has various manifestations and should be confirmed by clinical features,pathological features and imaging examination.The adult patients with multisystem and vital organ involvement suggest the poor prognosis.