TY - JOUR
T1 - The clinical and biological features of a series of immunophenotypic variant of B-CLL
AU - Cro, Lilla
AU - Ferrario, Andrea
AU - Lionetti, Marta
AU - Bertoni, Francesco
AU - Zucal N, Nadia
AU - Nobili, Lucia
AU - Fabris, Sonia
AU - Todoerti, Katia
AU - Cortelezzi, Agostino
AU - Guffanti, Andrea
AU - Goldaniga, Maria
AU - Marcheselli, Luigi
AU - Neri, Antonino
AU - Lambertenghi-Deliliers, Giorgio
AU - Baldini, Luca
PY - 2010/8
Y1 - 2010/8
N2 - Objectives: To describe the clinical and biological features of a series of immunophenotypic variant of B-CLL (v-CLL) characterised by intermediate RMH score, in the absence of t(11;14)(q13;q32) in FISH analysis in comparison with a series of typical CLL. Methods: We studied the clinical and biological features of 63 cases of v-CLL and 130 cases of CLL. Results: We observed significant differences in terms of age 9/L (P <0.001), lymphocyte doubling time ≤12 months (P = 0.02), high serum β2-microglobulin levels (P <0.001) and splenomegaly (P = 0.002); CD38, CD49d, CD1c were more expressed in v-CLL, CD43 in CLL (P <0.001). IgV
H mutation and trisomy 12 were more frequent in v-CLL group (P = 0.001; P <0.001); del13q14 in CLL (P = 0.008). Gene expression profiling of nine v-CLL and 60 CLL indicated that the atypical group presented a specific molecular pattern. After a median follow-up of respectively, 55 (4-196) and 60 months (6-180), 25/42 patients with v-CLL (48%) and 55/93 patients with CLL (59%) were treated. Time to treatment was significantly shorter in IgV
H-mutated v-CLL vs. mutated CLL (P = 0.006). The median overall survival was worse in v-CLL-mutated cases (P = 0.062). Conclusion: v-CLL should be identified and dealt with separately from classic CLL. In particular, the prognostic markers that are routinely used to characterise classical B-CLL should not be interpreted as having the same meaning.
AB - Objectives: To describe the clinical and biological features of a series of immunophenotypic variant of B-CLL (v-CLL) characterised by intermediate RMH score, in the absence of t(11;14)(q13;q32) in FISH analysis in comparison with a series of typical CLL. Methods: We studied the clinical and biological features of 63 cases of v-CLL and 130 cases of CLL. Results: We observed significant differences in terms of age 9/L (P <0.001), lymphocyte doubling time ≤12 months (P = 0.02), high serum β2-microglobulin levels (P <0.001) and splenomegaly (P = 0.002); CD38, CD49d, CD1c were more expressed in v-CLL, CD43 in CLL (P <0.001). IgV
H mutation and trisomy 12 were more frequent in v-CLL group (P = 0.001; P <0.001); del13q14 in CLL (P = 0.008). Gene expression profiling of nine v-CLL and 60 CLL indicated that the atypical group presented a specific molecular pattern. After a median follow-up of respectively, 55 (4-196) and 60 months (6-180), 25/42 patients with v-CLL (48%) and 55/93 patients with CLL (59%) were treated. Time to treatment was significantly shorter in IgV
H-mutated v-CLL vs. mutated CLL (P = 0.006). The median overall survival was worse in v-CLL-mutated cases (P = 0.062). Conclusion: v-CLL should be identified and dealt with separately from classic CLL. In particular, the prognostic markers that are routinely used to characterise classical B-CLL should not be interpreted as having the same meaning.
KW - B-cell chronic lymphocytic leukaemia
KW - FISH analysis
KW - IgV gene mutations
KW - immunophenotype
KW - ZAP-70 expression
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U2 - 10.1111/j.1600-0609.2010.01454.x
DO - 10.1111/j.1600-0609.2010.01454.x
M3 - Article
C2 - 20408870
AN - SCOPUS:77954719731
VL - 85
SP - 120
EP - 129
JO - European Journal of Haematology
JF - European Journal of Haematology
SN - 0902-4441
IS - 2
ER -