Clonal immunoglobulin light chain variable region germline gene use in AL amyloidosis: association with dominant amyloid‐related organ involvement and survival after stem cell transplantation

AL (primary or immunoglobulin light chain) amyloidosis (AL) differs from myeloma per se in that tissue deposits of amyloid are found, typically in association with small numbers of clonal plasma cells producing λ light chains, and also in that AL patients typically present with a predominantly dysfu...

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Bibliographic Details
Published in:British journal of haematology 1999-09, Vol.106 (3), p.744-751
Main Authors: Comenzo, Raymond L., Wally, Jeremy, Kica, Geraldina, Murray, Jessica, Ericsson, Thomas, Skinner, Martha, Zhang, Yana
Format: Article
Language:English
Subjects:
Adult
Aged
AL amyloidosis
Amyloidosis - genetics
Amyloidosis - therapy
Biological and medical sciences
Female
Hematology
Hematopoietic Stem Cell Transplantation - methods
Humans
Immunodeficiencies. Immunoglobulinopathies
immunoglobulin genes
immunoglobulin light chains
Immunoglobulin Variable Region - genetics
Immunoglobulinopathies
Immunopathology
Male
Medical sciences
Middle Aged
Plasma Cells
Reverse Transcriptase Polymerase Chain Reaction - methods
stem cell transplantation
Survival Analysis
Treatment Outcome
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Summary:AL (primary or immunoglobulin light chain) amyloidosis (AL) differs from myeloma per se in that tissue deposits of amyloid are found, typically in association with small numbers of clonal plasma cells producing λ light chains, and also in that AL patients typically present with a predominantly dysfunctional organ‐system. This constellation of features — fibrillar deposits comprised of light chains, λ light chain predominance, and organ‐system tropism and dysfunction — remains unexplained. Select patients with AL respond to haemopoietic stem cell transplantation (SCT) with clinical improvement and extended survival, particularly those who do not have cardiac involvement. In order to ascertain whether the organ‐system tropism of AL was associated with immunoglublin light chain variable region (Ig VL) germline gene utilization, we attempted to clone, sequence and assign germline donors to the clonal Ig VL genes of 62 AL patients, all of whom were treated with SCT. We succeeded in 39 cases, identifying clonal AL genes derived from donors of the λI (n = 10), λII (n = 5), λIII (n = 6), λVI (n = 11) and KI (n = 7) subtypes. The majority of the donors (IGLV6S1, DPL5, DPL2,DPL23 and LFVK431) were genes that appear in the expressed repertoire
ISSN:0007-1048
1365-2141
DOI:10.1046/j.1365-2141.1999.01591.x