Chronic lymphocytic leukemia with 6q− shows distinct hematological features and intermediate prognosis
Autor: | Maria Ciccone, Angelo Veronese, Maria Grazia Roberti, Paola Agostini, Gian Matteo Rigolin, Antonio Cuneo, M.G. Della Porta, Alessia Tieghi, Massimo Negrini, Luigi Cavazzini, Antonella Bardi, Elisa Tammiso, Gian Luigi Castoldi, Renato Bigoni, C De Angeli, Francesco Cavazzini |
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Rok vydání: | 2003 |
Předmět: |
Adult
Male Cancer Research medicine.medical_specialty Pathology Chronic lymphocytic leukemia Immunoglobulin Variable Region Biology Gene mapping Antigens CD hemic and lymphatic diseases Internal medicine medicine Humans ADP-ribosyl Cyclase Interphase In Situ Hybridization Fluorescence Aged Aged 80 and over Membrane Glycoproteins Hematology Middle Aged Prognosis medicine.disease ADP-ribosyl Cyclase 1 Leukemia Lymphocytic Chronic B-Cell Survival Rate Oncology Karyotyping Immunology Fish Chromosomes Human Pair 6 Female 6q Chromosome aberrations Chronic lymphotic leukemia FISH Immunoglobulin Heavy Chains |
Zdroj: | Leukemia. 18:476-483 |
ISSN: | 1476-5551 0887-6924 |
DOI: | 10.1038/sj.leu.2403242 |
Popis: | Cytogenetic and fluorescence in situ hybridization studies were successfully performed in 217 chronic lymphocytic leukemia (CLL). In all, 13 patients with 6q21 deletion were identified and characterized in comparison with 92 patients with 'favourable' karyotype (normal or 13q-), 69 cases with 'intermediate risk' (1-2 anomalies) and 43 cases with 'unfavourable' karyotype (complex, 11q- or 17p-). Six out of 13 cases with 6q- showed an excess of atypical lymphocytes, a finding confirmed at the histologic level;20% CD38+ cells were seen in 5/6 cases. IGVH mutational status revealed98% homology to the germline sequence in 4/10 cases. When compared with the 'favourable' group, patients with 6q- showed a higher white blood cell (WBC) count, frequent splenomegaly, atypical morphology, CD38+ and short time from diagnosis to first treatment and short survival. A higher median WBC count was found in the 6q- group vs the intermediate-risk group; survival was shorter in the unfavourable group. To ascertain if the 6q- anomaly was an independent factor predicting for an inferior outcome among those patients with 'favourable' cytogenetics, we performed an analysis of prognostic factors in 105 patients (92 'favourable' plus 13 with 6q-), showing that the 6q- chromosome maintained its prognostic significance at multivariate analysis (P=0.02) along with stage (P=0.01). We conclude that CLL with 6q- is characterized by a high incidence of atypical morphology, classical immunophenotype with CD38 positivity and intermediate incidence of IGVH somatic hypermutation. Clinicobiological features and outcome show that this cytogenetic subset of CLL should be allocated in an intermediate-risk category. |
Databáze: | OpenAIRE |
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