Τίτλος:
Clonal B-cell lymphocytosis exhibiting immunophenotypic features consistent with a marginal-zone origin: Is this a distinct entity?
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
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.
Συγγραφείς:
Xochelli, A.
Kalpadakis, C.
Gardiner, A.
Baliakas, P.
Vassilakopoulos, T.P.
Mould, S.
Davis, Z.
Stalika, E.
Kanellis, G.
Angelopoulou, M.K.
McIver-Brown, N.
Ibbotson, R.
Sachanas, S.
Korkolopoulou, P.
Athanasiadou, A.
Anagnostopoulos, A.
Papadaki, H.A.
Papadaki, T.
Stamatopoulos, K.
Pangalis, G.A.
Oscier, D.
Περιοδικό:
Blood advances
Εκδότης:
American Society of Hematology
Λέξεις-κλειδιά:
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
DOI:
10.1182/blood-2013-07-515155
