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Clinical and biological features of B-cell neoplasms with CDK6 translocations: an association with a subgroup of splenic marginal zone lymphomas displaying frequent CD5 expression, prolymphocytic cells, and TP53 abnormalities.
- Source :
-
British journal of haematology [Br J Haematol] 2021 Apr; Vol. 193 (1), pp. 72-82. Date of Electronic Publication: 2020 Dec 13. - Publication Year :
- 2021
-
Abstract
- A translocation involving the cyclin-dependent kinase 6 (CDK6) gene [t(CDK6)] is a rare but recurrent abnormality in B-cell neoplasms. To further characterise this aberration, we studied 57 cases; the largest series reported to date. Fluorescence in situ hybridisation analysis confirmed the involvement of CDK6 in all cases, including t(2;7)(p11;q21) immunoglobulin kappa locus (IGK)/CDK6 (n = 51), t(7;14)(q21;q32) CDK6/immunoglobulin heavy locus (IGH) (n = 2) and the previously undescribed t(7;14)(q21;q11) CDK6/T-cell receptor alpha locus (TRA)/T-cell receptor delta locus (TRD) (n = 4). In total, 10 patients were diagnosed with chronic lymphocytic leukaemia, monoclonal B-cell lymphocytosis or small lymphocytic lymphoma, and 47 had small B-cell lymphoma (SmBL) including 36 cases of marginal zone lymphoma (MZL; 34 splenic MZLs, one nodal MZL and one bronchus-associated lymphoid tissue lymphoma). In all, 18 of the 26 cytologically reviewed cases of MZL (69%) had an atypical aspect with prolymphocytic cells. Among the 47 patients with MZL/SmBL, CD5 expression was found in 26 (55%) and the tumour protein p53 (TP53) deletion in 22 (47%). The TP53 gene was mutated in 10/30 (33%); the 7q deletion was detected in only one case, and no Notch receptor 2 (NOTCH2) mutations were found. Immunoglobulin heavy-chain variable-region (IGHV) locus sequencing revealed that none harboured an IGHV1-02*04 gene. Overall survival was 82% at 10 years and not influenced by TP53 aberration. Our present findings suggest that most t(CDK6)+ neoplasms correspond to a particular subgroup of indolent marginal zone B-cell lymphomas with distinctive features.<br /> (© 2020 British Society for Haematology and John Wiley & Sons Ltd.)
- Subjects :
- Adult
Aged
Aged, 80 and over
Bronchial Neoplasms diagnosis
Bronchial Neoplasms metabolism
Cell Differentiation
Chromosome Aberrations
Female
Genes, p53 genetics
Humans
Immunoglobulin Heavy Chains metabolism
In Situ Hybridization, Fluorescence methods
Leukemia, Lymphocytic, Chronic, B-Cell diagnosis
Leukemia, Lymphocytic, Chronic, B-Cell drug therapy
Lymphoma, B-Cell, Marginal Zone diagnosis
Lymphoma, B-Cell, Marginal Zone drug therapy
Male
Middle Aged
Mutation
Phenotype
Survival Analysis
Tertiary Lymphoid Structures pathology
Translocation, Genetic genetics
Trisomy genetics
CD5 Antigens metabolism
Cyclin-Dependent Kinase 6 metabolism
Leukemia, Lymphocytic, Chronic, B-Cell metabolism
Lymphoma, B-Cell, Marginal Zone metabolism
Splenic Neoplasms pathology
Tumor Suppressor Protein p53 metabolism
Subjects
Details
- Language :
- English
- ISSN :
- 1365-2141
- Volume :
- 193
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- British journal of haematology
- Publication Type :
- Academic Journal
- Accession number :
- 33314017
- Full Text :
- https://doi.org/10.1111/bjh.17141