Letterer-Siwe Disease

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;

Details

Origin ID

C3160;

UMLS CUI

C0023381;

Automatic exact mappings (from CISMeF team)
- Langerhans-cell histiocytosis [DO Concept]
- Letterer-Siwe disease [ACR pathology]
- Letterer-Siwe disease [ACR pathology]
- Letterer-Siwe disease [ACR pathology]
- histiocytosis, Langerhans-Cell [MeSH Descriptor]
- histiocytosis, Langerhans-Cell [MeSH concept]
Currated CISMeF NLP mapping
- Familial Letterer-Siwe disease [MeSH concept]
- Langerhans cell histiocytosis, disseminated [ICD-O code]
- Langerhans cell histiocytosis, disseminated (disorder) [SNOMED CT concept]
- Langerhans cell histiocytosis, disseminated (morphologic abnormality) [SNOMED CT concept]
- Letterer Siwe disease [PASCAL descriptor]
- Letterer-Siwe disease [ICD-11 Extension code]
- Letterer-Siwe disease [ICD-11 Extension code]
- Letterer-Siwe disease [ICD-9 code]
- Letterer-Siwe disease [ICD-10 Sub-category (who)]
- Letterer-Siwe disease [Radlex concept]
- Letterer-Siwe disease [ORDO Disease]
- Letterer-Siwe disease [MedDRA LLT]
- Letterer-Siwe's disease [ICD-9 code]
- Letterer-siwe disease [OMIM Phenotype]
- Multifocal and multisystemic (disseminated) Langerhans-cell histiocytosis [ICD-10 Sub-category]
DO Cross reference
- Langerhans-cell histiocytosis [DO Concept]
Disease may have findings
- Bone Lesion [NCIt concept]
- CD68-Positive Neoplastic Cells Present [NCIt concept]
- Cutaneous Involvement [NCIt concept]
- Fever [NCIt concept]
- Hepatomegaly [NCIt concept]
- Lymphadenopathy [NCIt concept]
- Pancytopenia [NCIt concept]
- Sinusoidal Infiltration [NCIt concept]
- Splenomegaly [NCIt concept]
Has associated anatomic sites
- Hematopoietic and Lymphatic System [NCIt concept]
Semantic type(s)
- Disease or Syndrome [UMLS semantic type]
UMLS correspondences (same concept)
- Familial Letterer-Siwe disease [MeSH concept]
- Langerhans cell histiocytosis, disseminated [ICD-O code]
- Langerhans cell histiocytosis, disseminated (disorder) [SNOMED CT concept]
- Langerhans cell histiocytosis, disseminated (morphologic abnormality) [SNOMED CT concept]
- Letterer-Siwe disease [ORDO Disease]
- Letterer-Siwe disease [MedDRA LLT]
- Letterer-Siwe disease [ICD-9 code]
- Letterer-Siwe disease [Radlex concept]
- Letterer-Siwe disease [ICD-10 Sub-category (who)]
- Letterer-siwe disease [OMIM Phenotype]
- familial Letterer-Siwe disease [MeSH Supplementary Concept]
Validated automatic mappings to NTBT
- familial Letterer-Siwe disease [MeSH Supplementary Concept]
- langerhans cell histiocytosis [Disease (Nov.)]
concept_is_in_subset
- CPTAC Codelists Terminology [NCIt concept]
- CPTAC Neoplasms Codelist [NCIt concept]
- CPTAC Terminology [NCIt concept]
- 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]
- NCIt Neoplasm Core Terminology [NCIt concept]
- NICHD Terminology [NCIt concept]
- Pediatric Endocrinology Terminology [NCIt concept]
- Pediatric Hematology-Oncology Terminology [NCIt concept]
- mCode Cancer Disorder Value Set [NCIt concept]
- mCode Elixhauser Lymphoma Value Set [NCIt concept]
- mCode Primary Cancer Disorder Value Set [NCIt concept]
- mCode Primary Malignant Neoplasm Disorder Value Set [NCIt concept]
- mCode 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]
- Multifocal Multisystem Disease [NCIt concept]
- Polymorphic Cellular Infiltrate [NCIt concept]
- Rare Lesion [NCIt concept]
- S-100-Positive Neoplastic Cells Present [NCIt concept]
- Widespread Disease [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]

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