The clinical and biological features of a series of immunophenotypic variant of B-CLL.
OBJECTIVE
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).


CONCLUSIONS
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.
Cro
Lilla
L
UO Ematologia 1/CTMO, Università degli Studi, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy. cro@policlinico.mi.it
Ferrario
Andrea
A
Lionetti
Marta
M
Bertoni
Francesco
F
Zucal N
Nadia
N
Nobili
Lucia
L
Fabris
Sonia
S
Todoerti
Katia
K
Cortelezzi
Agostino
A
Guffanti
Andrea
A
Goldaniga
Maria
M
Marcheselli
Luigi
L
Neri
Antonino
A
Lambertenghi-Deliliers
Giorgio
G
Baldini
Luca
L
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D020869:Gene Expression Profiling; D015321:Gene Rearrangement; D006801:Humans; D007143:Immunoglobulin Heavy Chains; D016130:Immunophenotyping; D017404:In Situ Hybridization, Fluorescence; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000145:classification / Q000175:diagnosis / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D016019:Survival Analysis; D055815:Young Adult
D007143:Immunoglobulin Heavy Chains