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

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

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

Afiliación

Bomben R; Clinical and Experimental Hematology Research Unit, Centro di Riferimento Oncologico, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Aviano PN, Italy.

Blood ; 109(7): 2989-98, 2007 Apr 01.

Article en En | MEDLINE | ID: mdl-17148579

RESUMEN

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, among 122 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.

Asunto(s)

Cadenas Pesadas de Inmunoglobulina/genética; Región Variable de Inmunoglobulina/genética; Leucemia Linfocítica Crónica de Células B/genética; Leucemia Linfocítica Crónica de Células B/inmunología; Secuencia de Aminoácidos; Secuencia de Bases; Estudios de Casos y Controles; Cartilla de ADN/genética; Femenino; Perfilación de la Expresión Génica; Frecuencia de los Genes; Reordenamiento Génico de Cadena Pesada de Linfocito B; Genes de las Cadenas Pesadas de las Inmunoglobulinas; Humanos; Italia/epidemiología; Leucemia Linfocítica Crónica de Células B/mortalidad; Masculino; Persona de Mediana Edad; Datos de Secuencia Molecular; Mutación; Pronóstico; Homología de Secuencia de Aminoácido; Tasa de Supervivencia

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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Región Variable de Inmunoglobulina / Leucemia Linfocítica Crónica de Células B / Cadenas Pesadas de Inmunoglobulina Tipo de estudio: Clinical_trials / Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Female / Humans / Male / Middle aged País/Región como asunto: Europa Idioma: En Revista: Blood Año: 2007 Tipo del documento: Article País de afiliación: Italia Pais de publicación: Estados Unidos

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