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 <70 yr (P < 0.001), lymphocytosis <20 x 10(9)/L (P < 0.001), lymphocyte doubling time <or=12 months (P = 0.02), high serum beta2-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.