Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma with Unmutated Immunoglobulin Heavy Chain Variable-Region Gene

Preferred Label : Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma with Unmutated Immunoglobulin Heavy Chain Variable-Region Gene;

NCIt synonyms : Pregerminal Center Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma; CLL/SLL with Unmutated IGVH; Chronic Lymphocytic Leukemia Small Lymphocytic Lymphoma with Unmutated Immunoglobulin Heavy Chain-Region Gene;

NCIt definition : A recently recognized variant of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) that lacks somatic hypermutations of the Immunoglobulin heavy chain (IGH) genes, implying pregerminal center B-cell origin. Microarray gene expression profiling studies have demonstrated the expression of ZAP-70 gene (Syk family tyrosine kinase) in this subset of CLL/CLL. Patients with this variant of CLL/SLL have an unfavorable prognosis compared to those with somatic hypermutations of the IGH genes, with a median survival of approximately 6-8 years.;

Neoplastic status : Malignant;

Details

Origin ID

C37204;

UMLS CUI

C1333038;

Automatic exact mappings (from CISMeF team)
- pregerminal center chronic lymphocytic leukemia/small lymphocytic lymphoma [DO Concept]
DO Cross reference
- pregerminal center chronic lymphocytic leukemia/small lymphocytic lymphoma [DO Concept]
Disease excludes abnormal cell
- Neoplastic T-Lymphocyte and Neoplastic Natural Killer Cell [NCIt concept]
Disease excludes normal cell origin
- Myeloid Cell [NCIt concept]
- Precursor B-Lymphoblast [NCIt concept]
Disease may have findings
- Fatigue [NCIt concept]
- Lymphadenopathy [NCIt concept]
- Unfavorable Clinical Outcome [NCIt concept]
Has associated anatomic sites
- Hematopoietic and Lymphatic System [NCIt concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
UMLS correspondences (same concept)
- pregerminal center chronic lymphocytic leukemia/small lymphocytic lymphoma [DO Concept]
concept_is_in_subset
- NCIt Neoplasm Core Terminology [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 Lymphocyte [NCIt concept]
- Neoplastic Small Lymphocyte with Clumped Chromatin [NCIt concept]
disease_has_finding
- Malignant Cellular Infiltrate [NCIt concept]
disease_has_molecular_abnormality
- Clonal Immunoglobulin Gene Rearrangement [NCIt concept]
- Clonal Immunoglobulin Heavy Chain Gene Rearrangement [NCIt concept]
- Clonal Immunoglobulin Kappa Light Chain Gene Rearrangement [NCIt concept]
- Clonal Immunoglobulin Lambda Light Chain Gene Rearrangement [NCIt concept]
- Unmutated Immunoglobulin Heavy Chain Variable Region Gene [NCIt concept]
- ZAP70 Messenger RNA Overexpression [NCIt concept]
disease_has_normal_cell_origin
- B-Lymphocyte [NCIt concept]
- Hematopoietic and Lymphoid Cell [NCIt concept]
- Lymphocyte [NCIt concept]
- Mature B-Lymphocyte [NCIt concept]
- Naive Pregerminal Center 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_may_have_cytogenetic_abnormality
- del(11q22q23) [NCIt concept]
- del(17p13) [NCIt concept]

Keyword's position in hierarchy(ies) :

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