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

摘要: 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 its geographic distribution the context of country (Italy) both continental Mediterranean areas, 37 IGHV3-21 CLLs were collected out 1076 cases enrolled by different institutions from or Central Italy. Of cases, 18 identified as homologous (hom)HCDR3-IGHV3-21 found almost exclusively (16 18) Italy; contrast, 19 nonhomHCDR3-IGHV3-21 evenly distributed throughout Clinically, survivals documented for well subgroups mutated homHCDR3-IGHV3-21 CLLs. Negative prognosticators CD38, ZAP-70, CD49d, CD79b expressed at higher levels homHCDR3 cases. Differential expression profiling (GEP) 13 versus 52 non-IGHV3-21 identified, among 122 best-correlated genes, TGFB2 VIPR1 down- up-regulated CLL respectively. Moreover, GEP 7 6 yielded 20 differentially WNT-16 being that highest Altogether, CLLs, including those homHCDR3, had peculiar global phenotype part explaining their worse clinical outcome.