Stereotyped patterns of B-cell receptor in splenic marginal zone lymphoma

Haematologica. 2010 Oct;95(10):1792-6. doi: 10.3324/haematol.2010.025437. Epub 2010 May 29.


Antigen stimulation may be important for splenic marginal zone lymphoma pathogenesis. To address this hypothesis, the occurrence of stereotyped B-cell receptors was investigated in 133 SMZL (26 HCV+) compared with 4,414 HCDR3 sequences from public databases. Sixteen SMZL (12%) showed stereotyped BCR; 7 of 86 (8%) SMZL sequences retrieved from public databases also belonged to stereotyped HCDR3 subsets. Three categories of subsets were identified: i) "SMZL-specific subsets" (n=5), composed only of 12 SMZL (9 HCV-from our series); ii) "Non-Hodgkin's lymphoma-like subsets" (n=5), comprising 5 SMZL (4 from our series) clustering with other indolent lymphomas; iii) "CLL-like subsets" (n=6), comprising 6 SMZL (3 from our series) that belonged to known CLL subsets (n=4) or clustered with public CLL sequences. Immunoglobulin 3D modeling of 3 subsets revealed similarities in antigen binding regions not limited to HCDR3. Overall, data suggest that the pathogenesis of splenic marginal zone lymphoma may involve also HCV-unrelated epitopes or an antigenic trigger common to other indolent lymphomas.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Antigens
  • Epitopes
  • Gene Rearrangement
  • Humans
  • Immunoglobulin Heavy Chains / genetics
  • Lymphoma, B-Cell, Marginal Zone / etiology*
  • Lymphoma, B-Cell, Marginal Zone / immunology
  • Receptors, Antigen, B-Cell / immunology*
  • Splenic Neoplasms / etiology*
  • Splenic Neoplasms / immunology


  • Antigens
  • Epitopes
  • Immunoglobulin Heavy Chains
  • Receptors, Antigen, B-Cell