@article{3003923,
    title = "Clonal B-cell lymphocytosis exhibiting immunophenotypic features consistent with a marginal-zone origin: Is this a distinct entity?",
    author = "Xochelli, A. and Kalpadakis, C. and Gardiner, A. and Baliakas, P. and Vassilakopoulos, T.P. and Mould, S. and Davis, Z. and Stalika, E. and Kanellis, G. and Angelopoulou, M.K. and McIver-Brown, N. and Ibbotson, R. and Sachanas, S. and Korkolopoulou, P. and Athanasiadou, A. and Anagnostopoulos, A. and Papadaki, H.A. and Papadaki, T. and Stamatopoulos, K. and Pangalis, G.A. and Oscier, D.",
    journal = "Blood advances",
    year = "2014",
    volume = "123",
    number = "8",
    pages = "1199-1206",
    publisher = "American Society of Hematology",
    doi = "10.1182/blood-2013-07-515155",
    keywords = "CD5 antigen, adult;  aged;  article;  bone marrow biopsy;  cell infiltration;  chromosome 7q;  female;  follow up;  human;  human tissue;  immunogenetics;  immunophenotyping;  karyotype;  major clinical study;  male;  marginal zone lymphoma;  middle aged;  monoclonal b cell lymphocytosis;  priority journal;  somatic hypermutation;  splenomegaly;  very elderly, Adult;  Aged;  Aged, 80 and over;  B-Lymphocytes;  Cell Lineage;  Chromosome Banding;  Clone Cells;  Disease Progression;  Female;  Flow Cytometry;  Follow-Up Studies;  Gene Rearrangement, B-Lymphocyte, Heavy Chain;  Humans;  Immunophenotyping;  Lymphocytosis;  Lymphoma, B-Cell, Marginal Zone;  Male;  Middle Aged;  Retrospective Studies",
    abstract = "The biological and clinical significance of a clonal B-cell lymphocytosis with an immunophenotype consistent with marginal-zone origin (CBL-MZ) is poorly understood. We retrospectively evaluated 102 such cases with no clinical evidence to suggest a concurrent MZ lymphoma. Immunophenotyping revealed a clonal B-cell population with Matutes score ≤2 in all cases; 19/102 were weakly CD5 positive and all 35 cases tested expressed CD49d. Bone marrow biopsy exhibited mostly mixed patterns of small B-lymphocytic infiltration. A total of 48/66 (72.7%) cases had an abnormal karyotype. Immunogenetics revealed overusage of the IGHV4-34 gene and somatic hypermutation in 71/79 (89.8%) IGHV-IGHD-IGHJ gene rearrangements. With a median follow-up of 5 years, 85 cases remain stable (group A), whereas 17 cases (group B) progressed, of whom 15 developed splenomegaly. The clonal B-cell count, degree of marrow infiltration, immunophenotypic, or immunogenetic findings at diagnosis did not distinguish between the 2 groups. However, deletions of chromosome 7q were confined to group A and complex karyotypes were more frequent in group B. Although CBL-MZ may antedate SMZL/SLLU, most cases remain stable over time. These cases, not readily classifiable within the World Heath Organization classification, raise the possibility that CBL-MZ should be considered as a new provisional entity within the spectrum of clonal MZ disorders. © 2014 by The American Society of Hematology."
}