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

Autor: Daniela Marconi, Robin Foà, Pietro Bulian, Michele Dal Bo, Renato Campanini, Richard Rosenquist, Riccardo Bomben, Maria Ilaria Del Principe, Dimitar G. Efremov, Massimo Degan, Giovanni Del Poeta, Dania Benedetti, Mia Thorsélius, Anna Guarini, Francesco Forconi, Rossana Maffei, Giuseppe Palermo, Antonella Zucchetto, Gianluca Gaidano, Roberto Marasca, Emanuela M. Ghia, Luca Laurenti, Daniela Capello, Valter Gattei
Přispěvatelé: 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
Jazyk: angličtina
Rok vydání: 2007
Předmět:
Male
Chronic lymphocytic leukemia
Gene Rearrangement
B-Lymphocyte
Heavy Chain
Immunoglobulin Variable Region
Sequence Homology
CD38
Biochemistry
Gene Frequency
Humans
Prognosis
Amino Acid Sequence
Italy
Gene Expression Profiling
Base Sequence
Survival Rate
Leukemia
Lymphocytic
Chronic
B-Cell
DNA Primers
Molecular Sequence Data
Case-Control Studies
Middle Aged
Sequence Homology
Amino Acid
Genes
Immunoglobulin Heavy Chain
Immunoglobulin Heavy Chains
Mutation
Female
genetics
Chronic
genetics/immunology/mortality
Gene Rearrangement
Leukemia
B-Lymphocyte
IGHV3-21
CLL
Hematology
Phenotype
Lymphocytic
Amino Acid
medicine.anatomical_structure
epidemiology
Immunoglobulin gene
Immunoglobulin Heavy Chain
Immunology
Context (language use)
medicine
B cell
Amino Acid Sequence
Base Sequence
Case-Control Studies
DNA Primers
genetics
Female
Gene Expression Profiling
Gene Frequency
Gene Rearrangement
Heavy Chain
Genes
Immunoglobulin Heavy Chain
Humans
Immunoglobulin Heavy Chains
genetics
Immunoglobulin Variable Region
genetics
Italy
epidemiology
Leukemia
B-Cell
genetics/immunology/mortality
Male
Middle Aged
Molecular Sequence Data
Mutation
Prognosis
Sequence Homology
Amino Acid
Survival Rate
business.industry
Cell Biology
CD79B
medicine.disease
Gene expression profiling
Genes
Heavy Chain
business
Settore MED/15 - Malattie del Sangue
Popis: 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.
Databáze: OpenAIRE
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