Langerhans Cell Histiocytosis

Preferred Label : Langerhans Cell Histiocytosis;

NCIt synonyms : Histiocytosis X; Langerhans Cell Granulomatosis; LCH;

NCIt related terms : Langerhans cell histiocytosis, NOS;

NCIt definition : A neoplastic proliferation of Langerhans cells which contain Birbeck granules by ultrastructural examination. Three major overlapping syndromes are recognized: eosinophilic granuloma, Letterer-Siwe disease, and Hand-Schuller-Christian disease. The clinical course is generally related to the number of organs affected at presentation. (WHO, 2001);

Alternative definition : NCI-GLOSS: A group of rare disorders in which too many Langerhans cells (a type of white blood cell) grow in certain tissues and organs including the bones, skin, and lungs, and damage them. Langerhans cell histiocytosis may also affect the pituitary gland (which makes hormones that control other glands and many body functions, especially growth). Langerhans cell histiocytosis is most common in children and young adults.; NICHD: Tissue infiltration by Langerhans cells (histiocytes). When infiltration involves the pituitary stalk or gland, presentation includes diabetes insipidus, which is sometimes accompanied by other pituitary dysfunction.;

Neoplastic status : Undetermined;

ICD-O code : 9751/1; 9754/3; 9752/1; 9751/3; 9753/1;

Codes from synonyms : CDR0000513054; 10025581; CDR0000471787;

Details

Origin ID

C3107;

UMLS CUI

C0019621;

Automatic exact mappings (from CISMeF team)
- Langerhans cell histiocytosis [ICD-11 Extension code]
- Langerhans cell histiocytosis [ICD-11 Subcategory]
- Lissencephaly with cerebellar hypoplasia [ORDO Disease]
- Non langerhans cell histiocytosis [PASCAL descriptor]
- hip dislocation, congenital [MeSH Descriptor]
- histiocytosis, non-langerhans-cell [MeSH Descriptor]
Currated CISMeF NLP mapping
- Langerhans cell histiocytosis [PASCAL descriptor]
- Langerhans cell histiocytosis [Radlex concept]
- Langerhans cell histiocytosis [OMIM Phenotype]
- Langerhans cell histiocytosis [ORDO Disease]
- Langerhans cell histiocytosis (disorder) [SNOMED CT concept]
- Langerhans cell histiocytosis, NOS [ICD-O code]
- Langerhans cell histiocytosis, no International Classification of Diseases for Oncology subtype (morphologic abnormality) [Inactive SNOMED CT concept]
- Langerhans' cell granulomatosis [MedDRA LLT]
- Langerhans' cell histiocytosis [MedDRA Preferred Term]
- Langerhans-cell histiocytosis [DO Concept]
- histiocytosis, Langerhans-Cell [MeSH Descriptor]
- histiocytosis, Langerhans-Cell [MeSH concept]
- langerhans cell histiocytosis [Disease (Nov.)]
DO Cross reference
- Langerhans-cell histiocytosis [DO Concept]
Disease may have findings
- CD68-Positive Neoplastic Cells Present [NCIt concept]
- Sinusoidal Infiltration [NCIt concept]
Has associated anatomic sites
- Hematopoietic and Lymphatic System [NCIt concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
UMLS correspondences (same concept)
- Chronic idiopathic xanthomatosis [MedDRA LLT]
- Hand Schuller Christian disease [PASCAL descriptor]
- Hand-Schueller-Christian disease [MedDRA LLT]
- Hand-Schuller-Christian disease [Radlex concept]
- Hand-Schüller-Christian disease [ORDO Disease]
- Hand-Schüller-Christian disease (disorder) [SNOMED CT concept]
- Histiocytosis X syndrome (disorder) [SNOMED CT concept]
- Langerhans cell histiocytosis [ORDO Disease]
- Langerhans cell histiocytosis [OMIM Phenotype]
- Langerhans cell histiocytosis [Radlex concept]
- Langerhans cell histiocytosis [PASCAL descriptor]
- Langerhans cell histiocytosis (disorder) [SNOMED CT concept]
- Langerhans cell histiocytosis, NOS [ICD-O code]
- Langerhans cell histiocytosis, multifocal (morphologic abnormality) [SNOMED CT concept]
- Langerhans cell histiocytosis, no International Classification of Diseases for Oncology subtype (morphologic abnormality) [Inactive SNOMED CT concept]
- Langerhans' cell granulomatosis [MedDRA LLT]
- Langerhans' cell histiocytosis [MedDRA Preferred Term]
- Langerhans' cell histiocytosis (morphologic abnormality) [SNOMED CT concept]
- Langerhans-cell histiocytosis [DO Concept]
- hand-schuller-christian disease [SNOMED Notion]
- histiocytosis, Langerhans-Cell [MeSH concept]
- histiocytosis, Langerhans-Cell [MeSH Descriptor]
- langerhans cell histiocytosis [Disease (Nov.)]
concept_is_in_subset
- CTRP Disease Terminology [NCIt concept]
- CTRP Terminology [NCIt concept]
- Cellosaurus Disease Terminology [NCIt concept]
- Cellosaurus Terminology [NCIt concept]
- GDC Terminology [NCIt concept]
- GDC Value Terminology [NCIt concept]
- Mapped ICDO3.1 Morphology PT Terminology [NCIt concept]
- Mapped ICDO3.1 Morphology Terminology [NCIt concept]
- Mapped ICDO3.1 Terminology [NCIt concept]
- Mapped ICDO3.2 Morphology PT Terminology [NCIt concept]
- Mapped ICDO3.2 Morphology Terminology [NCIt concept]
- Mapped ICDO3.2 Terminology [NCIt concept]
- NCI CTEP SDC Hematopoietic Neoplasm, Miscellaneous Sub-Category Terminology [NCIt concept]
- NCI CTEP Simplified Disease Classification Terminology [NCIt concept]
- NCIt Neoplasm Core Terminology [NCIt concept]
- NICHD Terminology [NCIt concept]
- Pediatric Endocrinology Terminology [NCIt concept]
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]
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]
disease_may_have_molecular_abnormality
- BRAF Gene Mutation [NCIt concept]

Keyword's position in hierarchy(ies) :

You can consult :

recommendations
documents concerning education
documents for patients