Human B lymphocyte malignancies: exploitation of BLyS and APRIL and their receptors

Curr Dir Autoimmun. 2005;8:266-88. doi: 10.1159/000082107.


B lymphocyte stimulator (BLyS), also referred to as B cell activating factor of the TNF family, is a recently identified tumor necrosis factor (TNF) family member shown to be critical for maintenance of normal B cell development and homeostasis and it shares significant homology with another TNF superfamily member, a proliferation inducing ligand (APRIL). Currently, three receptors have been identified that transmit signals upon BLyS and APRIL binding and these include B cell maturation antigen, B cell activating factor receptor, and transmembrane activator and CAML interactor. The striking effects of BLyS on normal B cell maintenance and survival and the largely B lineage-restricted pattern of receptor expression, raises the possibility that these TNF family ligands and receptors may be involved not only in B cell autoimmunity, but also in the pathogenesis and maintenance of mature B lineage hematological malignancies. In this article, we will review the spectrum of human B lineage malignancies and discuss current evidence supporting a role for BLyS and APRIL in fueling the growth and survival of specific B cell malignancies.

Publication types

  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • B-Cell Activating Factor
  • B-Lymphocytes / immunology
  • B-Lymphocytes / pathology
  • Cell Differentiation
  • Humans
  • Leukemia, B-Cell / immunology*
  • Leukemia, B-Cell / pathology
  • Lymphoma, B-Cell / immunology*
  • Lymphoma, B-Cell / pathology
  • Membrane Proteins / immunology*
  • Models, Immunological
  • Multiple Myeloma / immunology
  • Receptors, Tumor Necrosis Factor / immunology
  • Tumor Necrosis Factor Ligand Superfamily Member 13
  • Tumor Necrosis Factor-alpha / immunology*


  • B-Cell Activating Factor
  • BLyS receptor
  • Membrane Proteins
  • Receptors, Tumor Necrosis Factor
  • TNFSF13 protein, human
  • TNFSF13B protein, human
  • Tumor Necrosis Factor Ligand Superfamily Member 13
  • Tumor Necrosis Factor-alpha