| Autor: |
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. |
| Jazyk: |
angličtina |
| Rok vydání: |
2014 |
| Popis: |
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. |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
|
