Pulmonary Langerhans Cell Histiocytosis

Preferred Label : Pulmonary Langerhans Cell Histiocytosis;

NCIt synonyms : Pulmonary Histiocytosis X; PLCH; Pulmonary Eosinophilic Granuloma;

NCIt definition : Langerhans cell histiocytosis affecting the lungs. It is characterized by an interstitial proliferation of Langerhans cells in the lung parenchyma. Most patients are current or former smokers. Steroids are the mainstay therapy for this condition.;

Neoplastic status : Undetermined;

Details

Origin ID

C142833;

UMLS CUI

C1455705;

Currated CISMeF NLP mapping
- Adult pulmonary Langerhans cell histiocytosis [ORDO Disease]
- Histiocytosis X of lung [MedDRA LLT]
- Langerhans cell histiocytosis of lung (disorder) [SNOMED CT concept]
- Pulmonary Langerhans cell histiocytosis [ICD-11 Extension code]
- Pulmonary eosinophilic granuloma [PASCAL descriptor]
- Pulmonary eosinophilic granuloma (disorder) [SNOMED CT concept]
- pulmonary eosinophilic granuloma [SNOMED Notion]
- pulmonary histiocytosis X [MeSH concept]
Disease may have findings
- CD68-Positive Neoplastic Cells Present [NCIt concept]
- Sinusoidal Infiltration [NCIt concept]
Excludes anatomical site(s)
- Neck [NCIt concept]
Has associated anatomic sites
- Hematopoietic and Lymphatic System [NCIt concept]
- Lung [NCIt concept]
- Respiratory System [NCIt concept]
- Thoracic Cavity [NCIt concept]
Semantic type(s)
- Disease or Syndrome [UMLS semantic type]
UMLS correspondences (same concept)
- Adult pulmonary Langerhans cell histiocytosis [ORDO Disease]
- Histiocytosis X of lung [MedDRA LLT]
- Langerhans cell histiocytosis of lung (disorder) [SNOMED CT concept]
- Pulmonary eosinophilic granuloma (disorder) [SNOMED CT concept]
- pulmonary eosinophilic granuloma [SNOMED Notion]
- pulmonary histiocytosis X [MeSH concept]
Validated automatic mappings to NTBT
- histiocytosis, Langerhans-Cell [MeSH Descriptor]
disease_excludes_finding
- CD21-Positive Neoplastic Cells Present [NCIt concept]
- CD30-Positive Neoplastic Cells Present [NCIt concept]
- CD35-Positive Neoplastic Cells Present [NCIt concept]
- HMB-45-Positive Neoplastic Cells Present [NCIt concept]
- High Grade Lesion [NCIt concept]
disease_excludes_molecular_abnormality
- Clonal Immunoglobulin Gene Rearrangement [NCIt concept]
- Clonal T-Cell Receptor Gene Rearrangement [NCIt concept]
disease_has_abnormal_cell
- Neoplastic Cell [NCIt concept]
- Neoplastic Hematopoietic and Lymphoid Cell [NCIt concept]
- Neoplastic Histiocytic and Dendritic Cell [NCIt concept]
- Neoplastic Langerhans Cell [NCIt concept]
disease_has_finding
- Birbeck Granules Present [NCIt concept]
- CD1a-Positive Neoplastic Cells Present [NCIt concept]
- Histiocytosis [NCIt concept]
- Monoclonal Langerhans Cells Present [NCIt concept]
- Polymorphic Cellular Infiltrate [NCIt concept]
- Rare Lesion [NCIt concept]
- S-100-Positive Neoplastic Cells Present [NCIt concept]
- Unifocal Lesion [NCIt concept]
disease_has_normal_cell_origin
- Hematopoietic and Lymphoid Cell [NCIt concept]
- Histiocyte [NCIt concept]
- Histiocytic and Dendritic Cell [NCIt concept]
- Langerhans Cell [NCIt concept]
disease_has_normal_tissue_origin
- Hematopoietic and Lymphoid Tissue [NCIt concept]
disease_has_primary_anatomic_site
- Hematopoietic and Lymphatic System [NCIt concept]
- Lung [NCIt concept]
disease_may_have_associated_disease
- Bronchiolitis [NCIt concept]
- Pulmonary Emphysema [NCIt concept]
disease_may_have_molecular_abnormality
- BRAF Gene Mutation [NCIt concept]

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