TY - JOUR
TI - Clonal B-cell lymphocytosis exhibiting immunophenotypic features consistent with a marginal-zone origin: Is this a distinct entity?
AU - Xochelli, A.
AU - Kalpadakis, C.
AU - Gardiner, A.
AU - Baliakas, P.
AU - Vassilakopoulos, T.P.
AU - Mould, S.
AU - Davis, Z.
AU - Stalika, E.
AU - Kanellis, G.
AU - Angelopoulou, M.K.
AU - McIver-Brown, N.
AU - Ibbotson, R.
AU - Sachanas, S.
AU - Korkolopoulou, P.
AU - Athanasiadou, A.
AU - Anagnostopoulos, A.
AU - Papadaki, H.A.
AU - Papadaki, T.
AU - Stamatopoulos, K.
AU - Pangalis, G.A.
AU - Oscier, D.
JO - Blood advances
PY - 2014
VL - 123
TODO - 8
SP - 1199-1206
PB - American Society of Hematology
SN - null
TODO - 10.1182/blood-2013-07-515155
TODO - 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
TODO - 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.
ER -