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

S. Zibellini, D. Capello, F. Forconi, P. Marcatili, D. Rossi, S. Rattotti, S. Franceschetti, E. Sozzi, E. Cencini, R. Marasca, L. Baldini, A. Tucci, F. Bertoni, F. Passamonti, E. Orlandi, M. Varettoni, M. Merli, S. Rizzi, V. Gattei, A. TramontanoM. Paulli, G. Gaidano, L. Arcaini

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72 Scopus citations


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. ©2010 Ferrata Storti Foundation.
Original languageEnglish (US)
Pages (from-to)1792-1796
Number of pages5
Issue number10
StatePublished - May 29 2010
Externally publishedYes

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