Targeting B-cell anergy in chronic lymphocytic leukemia

Blood. 2013 May 9;121(19):3879-88, S1-8. doi: 10.1182/blood-2012-12-474718. Epub 2013 Mar 4.

Abstract

B-cell receptor (BCR) triggering and responsiveness have a crucial role in the survival and expansion of chronic lymphocytic leukemia (CLL) clones. Analysis of in vitro response of CLL cells to BCR triggering allowed the definition of 2 main subsets of patients and lack of signaling capacity was associated with constitutive activation of extracellular-regulated kinases 1/2 (ERK1/2) and nuclear factor of activated T cells c1 (NF-ATc1), consistent with the idea that at least one group of CLL patients derives from the abnormal expansion of anergic B cells. In the present work, we further investigated the anergic subset of CLL (defined as the one with constitutive ERK1/2 phosphorylation) and found that it is characterized by low levels of surface immunoglobulin M and impairment of calcium mobilization after BCR engagement in vitro. Chronic BCR triggering promoted CLL cell survival selectively in phosphorylated ERK1/2 samples and the use of mitogen-activated protein kinase and NF-AT signaling inhibitors specifically induced apoptosis in this group of patients. Apoptosis induction was preceded by an initial phase of anergy reversal consisting in the loss of ERK phosphorylation and NF-AT nuclear translocation and by the restoration of BCR responsiveness, reinforcing the idea that the anergic program favors the survival of leukemic lymphocytes.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Antineoplastic Agents / therapeutic use
  • B-Lymphocytes* / immunology
  • B-Lymphocytes* / metabolism
  • B-Lymphocytes* / physiology
  • Clonal Anergy / immunology*
  • DNA-Binding Proteins / genetics
  • Female
  • Humans
  • Immunoglobulin gamma-Chains / genetics
  • Leukemia, Lymphocytic, Chronic, B-Cell / blood
  • Leukemia, Lymphocytic, Chronic, B-Cell / immunology
  • Leukemia, Lymphocytic, Chronic, B-Cell / therapy*
  • Mice
  • Mice, Knockout
  • Molecular Targeted Therapy / methods*
  • Receptors, Antigen, B-Cell / genetics
  • Receptors, Antigen, B-Cell / metabolism
  • Receptors, Antigen, B-Cell / physiology
  • Signal Transduction / genetics
  • Signal Transduction / immunology
  • Tumor Cells, Cultured

Substances

  • Antineoplastic Agents
  • DNA-Binding Proteins
  • Immunoglobulin gamma-Chains
  • Rag2 protein, mouse
  • Receptors, Antigen, B-Cell