research article
Langerhans cell histiocytosis
Abstract
Tumours derived from Langerhans cells (LCs) are divided into two main subgroups, according to the degree of cytological atypia and clinical aggressiveness: LC histiocytosis (LCH) and LC sarcoma. Both subgroups maintain the phenotypic profile and ultrastructural features of LCs. LCH is a clonal neoplastic proliferation of Langerhans-type cells that express CD1a, langerin, and S100 protein, showing Birbeck granules by ultrastructural examination. Here the clinicopathological of LCH will be discussed- Article
- Langerhans cell histiocytosis
- CD1a
- immunophenotype
- Cytogenetics
- Leukaemia Section
- Adult
- Bronchi/pathology
- Female
- Histiocytosis, Langerhans-Cell/*diagnostic imaging/*pathology/therapy
- Humans
- Lung/pathology
- Male
- Risk Factors
- Smoking/adverse effects
- Tomography, Spiral Computed
- Adolescent
- Adrenal Cortex Hormones/administration & dosage
- Antineoplastic Combined Chemotherapy Protocols/pharmacology/*therapeutic use
- Child
- Child, Preschool
- Cohort Studies
- Drug Resistance, Neoplasm
- Female
- France/epidemiology
- Histiocytosis, Langerhans-Cell/*drug therapy/enzymology/epidemiology/*genetics
- Humans