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Medline ® Abstract for Reference 46

of 'Clinical manifestations, pathologic features, and diagnosis of splenic marginal zone lymphoma'

46
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Clonal B-cell lymphocytosis exhibiting immunophenotypic features consistent with a marginal-zone origin: is this a distinct entity?
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Xochelli A, Kalpadakis C, Gardiner A, Baliakas P, Vassilakopoulos TP, Mould S, Davis Z, Stalika E, Kanellis G, Angelopoulou MK, McIver-Brown N, Ibbotson R, Sachanas S, Korkolopoulou P, Athanasiadou A, Anagnostopoulos A, Papadaki HA, Papadaki T, Stamatopoulos K, Pangalis GA, Oscier D
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Blood. 2014 Feb;123(8):1199-206. Epub 2013 Dec 3.
 
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.
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Hematology Department and HCT Unit, G. Papanicolaou Hospital, Thessaloniki, Greece;
PMID