Comprehensive characterization of IGHV3-21-expressing B-cell chronic lymphocytic leukemia: An Italian multicenter study

Riccardo Bomben, Michele Dal Bo, Daniela Capello, Dania Benedetti, Daniela Marconi, Antonella Zucchetto, Francesco Forconi, Rossana Maffei, Emanuela M. Ghia, Luca Laurenti, Pietro Bulian, Maria Ilaria Del Principe, Giuseppe Palermo, Mia Thorsélius, Massimo Degan, Renato Campanini, Anna Guarini, Giovanni Del Poeta, Richard Rosenquist, Dimitar G. EfremovRoberto Marasca, Robin Foà, Gianluca Gaidano, Valter Gattei

Research output: Contribution to journalArticlepeer-review


IGHV3-21-using chronic lymphocytic leukemia (CLL) is a distinct entity with restricted immunoglobulin gene features and poor prognosis and is more frequently encountered in Northern than Southern Europe. To further investigate this subset and its geographic distribution in the context of a country (Italy) with both continental and Mediterranean areas, 37 IGHV3-21 CLLs were collected out of 1076 cases enrolled by different institutions from Northern or Central Southern Italy. Of the 37 cases, 18 were identified as homologous (hom)HCDR3-IGHV3-21 CLLs and were found almost exclusively (16 of 18) in Northern Italy; in contrast, 19 nonhomHCDR3-IGHV3-21 cases were evenly distributed throughout Italy. Clinically, poor survivals were documented for IGHV3-21 CLLs as well as for subgroups of mutated and homHCDR3-IGHV3-21 CLLs. Negative prognosticators CD38, ZAP-70, CD49d, and CD79b were expressed at higher levels in homHCDR3 than nonhomHCDR3-IGHV3-21 cases. Differential gene expression profiling (GEP) of 13 IGHV3-21 versus 52 non-IGHV3-21 CLLs identified,among122 best-correlated genes, TGFB2 and VIPR1 as down- and up-regulated in IGHV3-21 CLL cases, respectively. Moreover, GEP of 7 homHCDR3 versus 6 nonhomHCDR3-IGHV3-21 CLLs yielded 20 differentially expressed genes, with WNT-16 being that expressed at the highest levels in homHCDR3-IGHV3-21 CLLs. Altogether, IGHV3-21 CLLs, including those with homHCDR3, had a peculiar global phenotype in part explaining their worse clinical outcome.

Original languageEnglish
Pages (from-to)2989-2998
Number of pages10
Issue number7
Publication statusPublished - Apr 1 2007

ASJC Scopus subject areas

  • Hematology


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