Preferred Label : Letterer-Siwe Disease;
NCIt related terms : Multifocal Multisystem Langerhans Cell Histiocytosis; Acute Disseminated Langerhans Cell Histiocytosis; Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis;
NCIt definition : A multifocal, multisystem form of Langerhans-cell histiocytosis. There is involvement
of multiple organ systems including the bones, skin, liver, spleen, and lymph nodes.
Patients are usually infants presenting with fever, hepatosplenomegaly, lymphadenopathy,
bone and skin lesions, and pancytopenia.;
Alternative definition : NICHD: A multifocal, multisystem form of Langerhans-cell histiocytosis. There is involvement
of multiple organ systems that may include the bones, skin, liver, spleen, and lymph
nodes. Patients are usually infants presenting with fever, hepatosplenomegaly, lymphadenopathy,
bone and skin lesions, and pancytopenia.;
Neoplastic status : Malignant;
ICD-O code : 9754/3;
Codes from synonyms : C96.0;
Origin ID : C3160;
UMLS CUI : C0023381;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
- DO Cross reference
- Disease may have findings
- Has associated anatomic sites
- Semantic type(s)
- UMLS correspondences (same concept)
- Validated automatic mappings to NTBT
- concept_is_in_subset
- disease_excludes_finding
- disease_excludes_molecular_abnormality
- disease_has_abnormal_cell
- disease_has_finding
- disease_has_normal_cell_origin
- disease_has_normal_tissue_origin
- disease_has_primary_anatomic_site
- disease_may_have_molecular_abnormality
