Increased BCR responsiveness in B cells from patients with chronic GVHD

Blood. 2014 Mar 27;123(13):2108-15. doi: 10.1182/blood-2013-10-533562. Epub 2014 Feb 14.

Abstract

Although B cells have emerged as important contributors to chronic graft-versus-host-disease (cGVHD) pathogenesis, the mechanisms responsible for their sustained activation remain unknown. We previously showed that patients with cGVHD have significantly increased B cell-activating factor (BAFF) levels and that their B cells are activated and resistant to apoptosis. Exogenous BAFF confers a state of immediate responsiveness to antigen stimulation in normal murine B cells. To address this in cGVHD, we studied B-cell receptor (BCR) responsiveness in 48 patients who were >1 year out from allogeneic hematopoietic stem cell transplantation (HSCT). We found that B cells from cGVHD patients had significantly increased proliferative responses to BCR stimulation along with elevated basal levels of the proximal BCR signaling components B cell linker protein (BLNK) and Syk. After initiation of BCR signaling, cGVHD B cells exhibited increased BLNK and Syk phosphorylation compared with B cells from patients without cGVHD. Blocking Syk kinase activity prevented relative post-HSCT BCR hyper-responsiveness of cGVHD B cells. These data suggest that a lowered BCR signaling threshold in cGVHD associates with increased B-cell proliferation and activation in response to antigen. We reveal a mechanism underpinning aberrant B-cell activation in cGVHD and suggest that therapeutic inhibition of the involved kinases may benefit these patients.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • B-Cell Activating Factor / metabolism
  • B-Lymphocytes / metabolism*
  • B-Lymphocytes / pathology
  • Cell Proliferation
  • Cells, Cultured
  • Chronic Disease
  • Female
  • Graft vs Host Disease / immunology*
  • Graft vs Host Disease / metabolism*
  • Humans
  • Lymphocyte Activation / physiology
  • Male
  • Middle Aged
  • Primary Cell Culture
  • Receptors, Antigen, B-Cell / agonists
  • Receptors, Antigen, B-Cell / physiology*
  • Young Adult

Substances

  • B-Cell Activating Factor
  • Receptors, Antigen, B-Cell