Large B-Cell Lymphoma with IRF4 Rearrangement

Preferred Label : Large B-Cell Lymphoma with IRF4 Rearrangement;

NCIt synonyms : LBCL with IRF4 Rearrangement;

NCIt definition : A large B-cell lymphoma that usually affects the Waldeyer ring and/or cervical lymph nodes and less often the gastrointestinal tract. It occurs most commonly in children and young adults and is low stage. It is characterized by a follicular, follicular and diffuse, or pure diffuse growth pattern resembling follicular lymphoma grade 3B or a diffuse large B-cell lymphoma. Most cases have IG/IRF4 rearrangements. BCL2 rearrangements are not present. Some cases that belong in this category lack an IRF4 rearrangement but have strong IRF4/MUM1 expression. This lymphoma is more aggressive than pediatric-type follicular lymphomas.;

Neoplastic status : Malignant;

NCI Metathesaurus CUI : CL521195;

Details

Origin ID

C133494;

UMLS CUI

C4524676;

Automatic exact mappings (from CISMeF team)
- Large B-cell lymphoma with IRF4 rearrangement [ICD-11 Extension code]
- Large B-cell lymphoma with interferon regulatory factor 4 rearrangement (morphologic abnormality) [SNOMED CT concept]
Disease excludes abnormal cell
- Neoplastic T-Lymphocyte and Neoplastic Natural Killer Cell [NCIt concept]
- Reed-Sternberg Cell [NCIt concept]
Disease excludes normal cell origin
- Myeloid Cell [NCIt concept]
- Plasma Cell [NCIt concept]
- Precursor B-Lymphoblast [NCIt concept]
Has associated anatomic sites
- Hematopoietic and Lymphatic System [NCIt concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
UMLS correspondences (same concept)
- Large B-cell lymphoma with interferon regulatory factor 4 rearrangement (morphologic abnormality) [SNOMED CT concept]
concept_is_in_subset
- CTRP Disease Terminology [NCIt concept]
- CTRP Terminology [NCIt concept]
disease_excludes_cytogenetic_abnormality
- t(14;18)(q32;q21) [NCIt concept]
disease_excludes_finding
- Benign Cellular Infiltrate [NCIt concept]
disease_has_abnormal_cell
- Abnormal Lymphocyte [NCIt concept]
- Malignant Cell [NCIt concept]
- Neoplastic B-Lymphocyte [NCIt concept]
- Neoplastic Cell [NCIt concept]
- Neoplastic Hematopoietic and Lymphoid Cell [NCIt concept]
- Neoplastic Large B-Lymphocyte [NCIt concept]
- Neoplastic Lymphocyte [NCIt concept]
disease_has_finding
- Malignant Cellular Infiltrate [NCIt concept]
- Nodular and Diffuse Pattern [NCIt concept]
disease_has_molecular_abnormality
- Clonal Immunoglobulin Gene Rearrangement [NCIt concept]
- Clonal Immunoglobulin Kappa Light Chain Gene Rearrangement [NCIt concept]
disease_has_normal_cell_origin
- B-Lymphocyte [NCIt concept]
- Germinal Center B-Lymphocyte [NCIt concept]
- Hematopoietic and Lymphoid Cell [NCIt concept]
- Lymphocyte [NCIt concept]
- Mature B-Lymphocyte [NCIt concept]
disease_has_normal_tissue_origin
- Hematopoietic and Lymphoid Tissue [NCIt concept]
- Lymphoid Tissue [NCIt concept]
disease_has_primary_anatomic_site
- Hematopoietic and Lymphatic System [NCIt concept]
- Lymphatic System [NCIt concept]
disease_mapped_to_gene
- IRF4 Gene [NCIt concept]
disease_may_have_molecular_abnormality
- IRF4 Gene Rearrangement [NCIt concept]
- IRF4/MUM1 Messenger RNA Overexpression [NCIt concept]
pathogenesis_of_disease_involves_gene
- IRF4 Gene [NCIt concept]
- IRF4 wt Allele [NCIt concept]

Keyword's position in hierarchy(ies) :

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