Role of BAFF in Opsoclonus-Myoclonus syndrome, a bridge between cancer and autoimmunity

Lizzia Raffaghello, Verena Fuhlhuber, Giovanna Bianchi, Massimo Conte, Franz Blaes, Claudio Gambini, Vito Pistoia

Research output: Contribution to journalArticlepeer-review


OMS is a rare paraneoplastic disorder that affects adults and children. Pediatric OMS is often associated with NB, a common, solid tumor of childhood, derived from the sympathetic nervous system. The detection of autoantibodies and lymphocytic infiltration in NB patients led to advance an autoimmune hypothesis for the pathogenesis of OMS-related NB. BAFF is a potent modulator of B cell growth and survival upon interaction with its receptors BAFF-R and BCMA. The aim of this study was to investigate mechanism(s) involved in ectopic lymphoid neogenesis in OMS-associated NB. We investigated BAFF, BAFF-R, and BCMA expression in NB tumors associated or not with OMS. Furthermore, we evaluated BAFF expression and secretion in NB cell lines, treated or untreated with differentiating agents. Immunohistochemically, lymphocytes infiltrating NB tumors from patients, with or without OMS, expressed BAFF, BAFF-R, and BCMA, whereas neuroblasts expressed BAFF and BCMA but not BAFF-R. By flow cytometry, BAFF was found to be consistently expressed in NB cell lines. Similarly to the results obtained in tissue lesions, BCMA but not BAFF-R was detected on the surface of all NB cell lines under basal conditions. De novo synthesis of BAFF-R and up-regulation of BCMA were observed in NB cell lines upon treatment with IFN-γ or 13-cis retinoic acid. This study provides new insights in the mechanisms driving the neogenesis of lymphoid follicles and in the functional interactions between tumor and immune cells in OMS-associated NB.

Original languageEnglish
Pages (from-to)183-191
Number of pages9
JournalJournal of Leukocyte Biology
Issue number1
Publication statusPublished - Jul 2013


  • B lymphocytes
  • Mechanisms of intratumor lymphoid neogenesis
  • Neuroblastoma
  • Paraneoplastic syndrome

ASJC Scopus subject areas

  • Cell Biology
  • Immunology


Dive into the research topics of 'Role of BAFF in Opsoclonus-Myoclonus syndrome, a bridge between cancer and autoimmunity'. Together they form a unique fingerprint.

Cite this