Burden of cytogenetically abnormal plasma cells in light chain amyloidosis and their prognostic relevance

Leuk Res. 2016 May:44:45-52. doi: 10.1016/j.leukres.2016.03.001. Epub 2016 Mar 10.

Abstract

We performed cytoplasmic fluorescence in situ hybridization assays of light chain amyloidosis (AL). In total, 234 patients were enrolled: 28 patients with AL, 24 with monoclonal gammopathy of undetermined significance (MGUS), and 182 with multiple myeloma (MM). Chromosomal abnormalities were detected in 13 of 22 (59%) AL patients without MM. All 13 patients demonstrated IGH rearrangement, and t(11;14)/IGH-CCND1 was most frequent (32%). Chromosome gain was not observed in AL patients without MM. These findings were dissimilar to findings in MGUS patients, in whom trisomy 9 was the most frequent abnormality. Of 6 AL patients with MM, 5 (83%) patients had cytogenetic abnormalities: 1q gain (4/6, 67%), gains of chromosome 9 (3/6, 50%), IGH rearrangement and RB1 (13q) deletions (2/6 each, 33%). The percentage of clonal plasma cells among total plasma cells was variable (median, 75%; range, 16-100%) for AL patients without MM, which was lower than the results for MM patients (median 100%). The overall survival of AL patients without MM was not significantly different according to the presence of cytogenetic abnormalities (P=0.510). In summary, among Korean AL patients, IGH rearrangement was the most frequent cytogenetic abnormality and cytogenetic aberration patterns differ compared with MGUS and MM patients.

Keywords: AL amyloidosis; Cytogenetics; Monoclonal gammopathy of undermined significance; Multiple myeloma.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Amyloidosis / genetics
  • Amyloidosis / mortality
  • Amyloidosis / pathology*
  • Chromosome Aberrations*
  • Chromosomes, Human, Pair 13 / genetics
  • Female
  • Follow-Up Studies
  • Humans
  • Immunoglobulin Light Chains / genetics*
  • In Situ Hybridization, Fluorescence
  • Male
  • Middle Aged
  • Monoclonal Gammopathy of Undetermined Significance / genetics
  • Monoclonal Gammopathy of Undetermined Significance / mortality
  • Monoclonal Gammopathy of Undetermined Significance / pathology*
  • Multiple Myeloma / genetics
  • Multiple Myeloma / mortality
  • Multiple Myeloma / pathology*
  • Neoplasm Staging
  • Plasma Cells / pathology*
  • Prognosis
  • Survival Rate

Substances

  • Immunoglobulin Light Chains