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Chronic Lymphocytic Leukemia With Two B-Cell Populations of Discordant Light Chain Restrictions in Individual Patients: Parallel Development of Biclonal B-Cell Neoplasms or Clonal Evolution With Isotype Switch?
- Source :
-
American journal of clinical pathology [Am J Clin Pathol] 2023 Apr 04; Vol. 159 (4), pp. 337-351. - Publication Year :
- 2023
-
Abstract
- Objectives: To evaluate clinicopathologic characteristics of biclonal chronic lymphocytic leukemia (CLL).<br />Methods: Retrospectively analyze clinical data and pathologic features.<br />Results: Ten cases were identified in which flow cytometry demonstrated an abnormal B-cell population with a CLL-like immunophenotype but showed no definitive light chain restriction. All had cytogenetic abnormalities detected, including seven with two CLL-related abnormalities. Four of these showed features suggestive of clonal evolution, all having del(13q) as a "stem-line" abnormality and three showing del(11q) as a "side-line" abnormality. Five (50%) cases demonstrated deleterious NOTCH1 mutations, in contrast to 11.8% in a control group of monoclonal CLL (Pā <ā .05). Of the 10 patients, 5 received treatment, with good/partial response in three cases and therapeutic resistance in one case. The median treatment-free survival was estimated at 68 months.<br />Conclusions: Despite a polytypic pattern of light chain expression, the neoplastic nature of biclonal CLL is suggested by a characteristic CLL phenotype and can be confirmed by cytogenetic and genomic analyses. The two clones with discordant light chain isotypes may share a "stem-line" cytogenetic abnormality, suggesting possible clonal evolution. Biclonal CLL is associated with NOTCH1 mutations, which may occur in a small subclone and gradually evolve in clonal size. Genomic analysis on light chain-sorted and/or chronologically collected samples may provide insight into clonal evolution in CLL.<br /> (© The Author(s) 2023. Published by Oxford University Press on behalf of American Society for Clinical Pathology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)
- Subjects :
- Chromosome Aberrations
Receptor, Notch1 genetics
Humans
Male
Female
Middle Aged
Aged
Chromosomes, Human, Pair 11 genetics
Chromosomes, Human, Pair 13
Leukemia, Lymphocytic, Chronic, B-Cell genetics
Leukemia, Lymphocytic, Chronic, B-Cell pathology
B-Lymphocytes pathology
Clonal Evolution
Immunoglobulin Light Chains genetics
Immunoglobulin Class Switching genetics
Subjects
Details
- Language :
- English
- ISSN :
- 1943-7722
- Volume :
- 159
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- American journal of clinical pathology
- Publication Type :
- Academic Journal
- Accession number :
- 36749322
- Full Text :
- https://doi.org/10.1093/ajcp/aqac165