Langerhans cell histiocytosis

Preferred Label : Langerhans cell histiocytosis;

CISMeF acronym : LCH;

Type : Other, mainly phenotypes with suspected mendelian basis;

Alternative titles and symbols : LCH;

Description : Langerhans cell histiocytosis (LCH) is a rare disorder characterized by the dysregulated proliferation of Langerhans cells and subsequent organ infiltration. Clinical manifestations range from a spontaneously healing isolated osteolytic lesion to a lymphoma-like syndrome with fatal multiorgan failure, in the absence of any cellular evidence of malignancy. Although the disease can present at any age, the peak age at diagnosis is between 1 and 3 years (summary by Arico et al., 1999). Egeler and D'Angio (1995) presented a classification of histiocytosis syndromes in children: class I, Langerhans cell histiocytosis (LCH); class II, histiocytosis of mononuclear macrophages other than Langerhans cells, including familial hemophagocytic lymphohistiocytosis (267700); and class III, malignant histiocytic disorders, including histiocytic lymphoma.;

Prefixed ID : 604856;

Details

Origin ID

604856;

UMLS CUI

C0019621;

Automatic exact mappings (from CISMeF team)
- Adult Langerhans Cell Histiocytosis [NCIt concept]
- Childhood Langerhans Cell Histiocytosis [NCIt concept]
- Langerhans cell histiocytosis [ICD-11 Subcategory]
- Lissencephaly co-occurrent with congenital cerebellar hypoplasia (disorder) [SNOMED CT concept]
- Non langerhans cell histiocytosis [PASCAL descriptor]
- hip dislocation, congenital [MeSH Descriptor]
- histiocytosis, non-langerhans-cell [MeSH Descriptor]
- lithocholyl-N-hydroxysuccinimide [MeSH concept]
- lithocholyl-N-hydroxysuccinimide [MeSH Supplementary Concept]
Currated CISMeF NLP mapping
- Langerhans Cell Histiocytosis [NCIt concept]
- Langerhans cell histiocytosis [PASCAL descriptor]
- Langerhans cell histiocytosis [Radlex concept]
- 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 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]
False automatic mappings
- Lissencephaly with cerebellar hypoplasia [ORDO Disease]
ORDO concept(s)
- Langerhans cell histiocytosis [ORDO Disease]
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 [NCIt concept]
- 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 [NCIt concept]
- Langerhans cell histiocytosis [ORDO Disease]
- 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 Descriptor]
- histiocytosis, Langerhans-Cell [MeSH concept]
- langerhans cell histiocytosis [Disease (Nov.)]

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