Diagnostic role and prognostic significance of a simplified immunophenotypic classification of mature B cell chronic lymphoid leukemias.
We verified the diagnostic and prognostic role of a simplified immunophenotypic classification (IC) in a series of 258 patients (M/F: 1.4; median age: 64 years; median follow-up: 64 months; 75 deaths) with mature B cell lymphoid leukemias (MBC-LL) for whom no histopathological diagnosis was available because of minimal or no lymph node involvement. The IC was based on the reactivity of three pivotal immunophenotypic markers: CD5, CD23 and SIg intensity. On the basis of different expression patterns, we identified four diagnostic clusters (C) characterized by distinct clinico-biological features and different prognoses: C1 (149 patients) identified most classical B cell chronic lymphocytic leukemias (CLL-type cluster; SIg(dim)/CD5+/CD23+); C2, 38 patients whose clinico-hematological characteristics were intermediate between C1 and C3 (CLL-variant cluster; SIg(bright)/CD5+/CD23+/-or SIg(dim)/CD5-/-/CD23 indifferent); C3 (16 patients) most situations consistent with mantle cell lymphoma in leukemic phase (MCL-type cluster; SIg(bright)/CD5+/CD23-); and C4, 55 cases, most of whom were consistent with leukemic phase lymphoplasmacytic/splenic marginal zone lymphomas (LP/S-type cluster; SIg(bright)/CD5-/+/CD23 indifferent). At univariate survival analysis, prognosis worsened from C1 to C4, C2 and C3 (P = 0.0001), and this was maintained at multivariate analysis (P = 0.006), together with CD11c expression (P = 0.0043), age at diagnosis (cut-off 70 years; P = 0.0008) and platelet count (cut-off 140 x 10(9)/l; P = 0.0034). Besides recognising the two well-known situations of classic B-CLL and MCL, our IC identified situations with distinct prognostic and/or clinical behaviors.
Cro
L
L
Unità Operativa Ematologia 1, Dipartimento di Ematologia, Ospedale Maggiore, IRCCS, Milan, Italy.
Guffanti
A
A
Colombi
M
M
Cesana
B
B
Grimoldi
M G
MG
Patriarca
C
C
Goldaniga
M
M
Neri
A
A
Intini
D
D
Cortelezzi
A
A
Maiolo
A T
AT
Baldini
L
L
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015703:Antigens, CD / Q000276:immunology; D015153:Blotting, Western; D018956:CD5 Antigens / Q000276:immunology*; D002869:Chromosome Aberrations; D004268:DNA-Binding Proteins / Q000235:genetics; D005260:Female; D005500:Follow-Up Studies; D015972:Gene Expression Regulation, Neoplastic; D019940:Genes, bcl-1 / Q000502:physiology; D006801:Humans; D007136:Immunoglobulins / Q000276:immunology / Q000378:metabolism; D016130:Immunophenotyping; D007621:Karyotyping; D037102:Lectins / Q000276:immunology*; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000145:classification* / Q000175:diagnosis* / Q000276:immunology; D008214:Lymphocytes / Q000097:blood / Q000378:metabolism; D008297:Male; D008565:Membrane Proteins / Q000276:immunology*; D008875:Middle Aged; D011518:Proto-Oncogene Proteins / Q000235:genetics; D051560:Proto-Oncogene Proteins c-bcl-6; D017455:Receptors, IgE / Q000276:immunology*; D012680:Sensitivity and Specificity; D015996:Survival Rate; D014157:Transcription Factors / Q000235:genetics
D015703:Antigens, CD; D018956:CD5 Antigens; D004268:DNA-Binding Proteins; D007136:Immunoglobulins; D037102:Lectins; D008565:Membrane Proteins; D011518:Proto-Oncogene Proteins; D051560:Proto-Oncogene Proteins c-bcl-6; D017455:Receptors, IgE; C432400:SIGLEC12 protein, human; D014157:Transcription Factors