Diagnostic role and prognostic significance of a simplified immunophenotypic classification of mature B cell chronic lymphoid leukemias

Leukemia. 2003 Jan;17(1):125-32. doi: 10.1038/sj.leu.2402737.

Abstract

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.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Antigens, CD / immunology
  • Blotting, Western
  • CD5 Antigens / immunology*
  • Chromosome Aberrations
  • DNA-Binding Proteins / genetics
  • Female
  • Follow-Up Studies
  • Gene Expression Regulation, Neoplastic*
  • Genes, bcl-1 / physiology
  • Humans
  • Immunoglobulins / immunology
  • Immunoglobulins / metabolism
  • Immunophenotyping
  • Karyotyping
  • Lectins / immunology*
  • Leukemia, Lymphocytic, Chronic, B-Cell / classification*
  • Leukemia, Lymphocytic, Chronic, B-Cell / diagnosis*
  • Leukemia, Lymphocytic, Chronic, B-Cell / immunology
  • Lymphocytes / blood
  • Lymphocytes / metabolism
  • Male
  • Membrane Proteins / immunology*
  • Middle Aged
  • Proto-Oncogene Proteins / genetics
  • Proto-Oncogene Proteins c-bcl-6
  • Receptors, IgE / immunology*
  • Sensitivity and Specificity
  • Survival Rate
  • Transcription Factors / genetics

Substances

  • Antigens, CD
  • CD5 Antigens
  • DNA-Binding Proteins
  • Immunoglobulins
  • Lectins
  • Membrane Proteins
  • Proto-Oncogene Proteins
  • Proto-Oncogene Proteins c-bcl-6
  • Receptors, IgE
  • SIGLEC12 protein, human
  • Transcription Factors