Abstract
Immunoglobulin light chain amyloidosis (AL) is characterized by a limited clonal expansion of plasma cells and amyloid formation. Here, we report restriction in the diversity of VL gene usage with a dominance of clonally related B cells in the peripheral blood (PB) isotype-specific repertoire of AL patients. A rigorous quantification of lineage trees reveals presence of intraclonal variations in the PB clones compared to the bone marrow (BM) clones, which suggests a common precursor that is still subject to somatic mutation. When compared to normal BM and PB B cells, AL clones showed significant but incomplete impairment of antigenic selection, which could not be detected by conventional R and S mutation analysis. Therefore, graphical analysis of B cell lineage trees and mathematical quantification of tree properties provide novel insights into the process of B cell clonal evolution in AL.
Original language | English (US) |
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Pages (from-to) | 69-87 |
Number of pages | 19 |
Journal | Journal of Clinical Immunology |
Volume | 27 |
Issue number | 1 |
DOIs | |
State | Published - Jan 2007 |
Keywords
- B cells
- Cell differentiation
- Human
- Immunoglobulin light chain
- Light chain amyloidosis
- Plasma cells
- Repertoire development
ASJC Scopus subject areas
- Immunology and Allergy
- Immunology