Blood First Edition Paper, prepublished online December 5, 2006; DOI 10.1182/blood-2006-10-051110. IGHV3-21-using chronic lymphocytic leukemia (CLL) is a distinct entity with restricted immunoglobulin gene features and poor prognosis, more frequently encountered in Northern than Southern Europe. To further investigate this subset and its geographical 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. 18/37 cases, identified as homologous(hom)HCDR3-IGHV3-21 CLLs, were found almost exclusively (16/18) in Northern Italy, while 19 non-homHCDR3-IGHV3-21 cases were similarly 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, CD79b were expressed at higher levels in homHCDR3 than non-homHCDR3-IGHV3-21 cases. Differential gene expression profiling (GEP) of 13 IGHV3-21 vs. 52 non-IGHV3-21 CLLs, identified, among 122 best-correlated genes, TGFB2 and VIPR1 as down- and up-regulated in IGHV3-21 CLL cases, respectively. Moreover, GEP of 7 homHCDR3 vs. 6 non-homHCDR3-IGHV3-21 CLLs yielded 20 differentially expressed genes, WNT16 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.
Comprehensive characterization of IGHV3-21-expressing B-cell chronic lymphocytic leukemia: an Italian multicenter study / Comprehensive characterization of IGHV3-21-expressing B-cell chronic lymphocytic leukemia: an Italian multicenter study. R. Bomben ; M. Dal Bo ; D. Capello ; D. Benedetti ; D. Marconi ; A. Zucchetto ; F. Forconi ; R. Maffei ; E. Ghia ; L. Laurenti ; P.Bulian ; M.I. Del Principe ; G. Palermo ; M. Thorselius ; M. Degan ; R.Campanini ; A.Guarini ; G. Del Poeta ; R. Rosenquist ; D. G .Efremov ; R.Marasca ; R.Foa ; G. Gaidano ; V.Gattei. - In: BLOOD. - ISSN 0006-4971. - ELETTRONICO. - blood-2006-10-051110v1:(2006). [10.1182/blood-2006-10-051110]
Comprehensive characterization of IGHV3-21-expressing B-cell chronic lymphocytic leukemia: an Italian multicenter study.
CAMPANINI, RENATO;
2006
Abstract
Blood First Edition Paper, prepublished online December 5, 2006; DOI 10.1182/blood-2006-10-051110. IGHV3-21-using chronic lymphocytic leukemia (CLL) is a distinct entity with restricted immunoglobulin gene features and poor prognosis, more frequently encountered in Northern than Southern Europe. To further investigate this subset and its geographical 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. 18/37 cases, identified as homologous(hom)HCDR3-IGHV3-21 CLLs, were found almost exclusively (16/18) in Northern Italy, while 19 non-homHCDR3-IGHV3-21 cases were similarly 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, CD79b were expressed at higher levels in homHCDR3 than non-homHCDR3-IGHV3-21 cases. Differential gene expression profiling (GEP) of 13 IGHV3-21 vs. 52 non-IGHV3-21 CLLs, identified, among 122 best-correlated genes, TGFB2 and VIPR1 as down- and up-regulated in IGHV3-21 CLL cases, respectively. Moreover, GEP of 7 homHCDR3 vs. 6 non-homHCDR3-IGHV3-21 CLLs yielded 20 differentially expressed genes, WNT16 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.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
