A mutated B cell chronic lymphocytic leukemia subset that recognizes and responds to fungi

J Exp Med. 2013 Jan 14;210(1):59-70. doi: 10.1084/jem.20121801. Epub 2013 Jan 7.

Abstract

B cell chronic lymphocytic leukemia (CLL), the most common leukemia in adults, is a clonal expansion of CD5(+)CD19(+) B lymphocytes. Two types of CLLs are being distinguished as carrying either unmutated or somatically mutated immunoglobulins (Igs), which are associated with unfavorable and favorable prognoses, respectively. More than 30% of CLLs can be grouped based on their expression of stereotypic B cell receptors (BCRs), strongly suggesting that distinctive antigens are involved in the development of CLL. Unmutated CLLs, carrying Ig heavy chain variable (IGHV) genes in germline configuration, express low-affinity, poly-, and self-reactive BCRs. However, the antigenic specificity of CLLs with mutated IGHV-genes (M-CLL) remained elusive. In this study, we describe a new subset of M-CLL, expressing stereotypic BCRs highly specific for β-(1,6)-glucan, a major antigenic determinant of yeasts and filamentous fungi. β-(1,6)-glucan binding depended on both the stereotypic Ig heavy and light chains, as well as on a distinct amino acid in the IGHV-CDR3. Reversion of IGHV mutations to germline configuration reduced the affinity for β-(1,6)-glucan, indicating that these BCRs are indeed affinity-selected for their cognate antigen. Moreover, CLL cells expressing these stereotypic receptors proliferate in response to β-(1,6)-glucan. This study establishes a class of common pathogens as functional ligands for a subset of somatically mutated human B cell lymphomas.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aspergillus / metabolism
  • B-Lymphocytes / cytology
  • B-Lymphocytes / metabolism
  • Candida / metabolism
  • Case-Control Studies
  • Cell Proliferation
  • Epitopes / metabolism*
  • Humans
  • Immunoglobulin Heavy Chains / genetics
  • Immunoglobulin M / genetics
  • Immunoglobulin M / metabolism
  • Immunoglobulin Variable Region / genetics
  • Leukemia, Lymphocytic, Chronic, B-Cell / genetics*
  • Leukemia, Lymphocytic, Chronic, B-Cell / immunology
  • Leukemia, Lymphocytic, Chronic, B-Cell / pathology
  • Mutation
  • Receptors, Antigen, B-Cell / genetics*
  • Receptors, Antigen, B-Cell / metabolism*
  • Recombinant Proteins / genetics
  • Recombinant Proteins / metabolism
  • Saccharomyces / metabolism
  • Trichosporon / metabolism
  • Yeasts / metabolism*
  • beta-Glucans / immunology
  • beta-Glucans / metabolism*

Substances

  • Epitopes
  • Immunoglobulin Heavy Chains
  • Immunoglobulin M
  • Immunoglobulin Variable Region
  • Receptors, Antigen, B-Cell
  • Recombinant Proteins
  • beta-Glucans
  • beta-1,6-glucan