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Leukemia. 2004 Mar;18(3):476-83.
Chronic lymphocytic leukemia with 6q-
shows distinct hematological features and
intermediate prognosis.
Cuneo A,
Rigolin GM, Bigoni R, De Angeli C, Veronese A,
Cavazzini F, Bardi A, Roberti MG, Tammiso E,
Agostini P, Ciccone M, Della Porta M, Tieghi A,
Cavazzini L, Negrini M, Castoldi G. Dipartimento di
Scienze Biomediche e Terapie Avanzate, Sezione di
Ematologia, Ferrara, Italy. sse@dns.unife.it
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 revealed >98% 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.
PMID: 14712287 [PubMed - indexed for
MEDLINE]