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Clonal B-cell lymph...
Clonal B-cell lymphocytosis exhibiting immunophenotypic features consistent with a marginal-zone origin : is this a distinct entity?
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Xochelli, Aliki (author)
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Kalpadakis, Christina (author)
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Gardiner, Anne (author)
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- Baliakas, Panagiotis (author)
- Uppsala universitet,Hematologi och immunologi
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Vassilakopoulos, Theodoros P. (author)
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Mould, Sarah (author)
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Davis, Zadie (author)
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Stalika, Evangelia (author)
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Kanellis, George (author)
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Angelopoulou, Maria K. (author)
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McIver-Brown, Neil (author)
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Ibbotson, Rachel (author)
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Sachanas, Sotirios (author)
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Korkolopoulou, Penelope (author)
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Athanasiadou, Anastasia (author)
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Anagnostopoulos, Achilles (author)
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Papadaki, Helen A. (author)
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Papadaki, Theodora (author)
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- Stamatopoulos, Kostas (author)
- Uppsala universitet,Institutionen för immunologi, genetik och patologi
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Pangalis, Gerassimos A. (author)
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Oscier, David (author)
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(creator_code:org_t)
- American Society of Hematology, 2014
- 2014
- English.
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In: Blood. - : American Society of Hematology. - 0006-4971 .- 1528-0020. ; 123:8, s. 1199-1206
- Related links:
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https://urn.kb.se/re...
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https://doi.org/10.1...
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Abstract
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- 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.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)
Publication and Content Type
- ref (subject category)
- art (subject category)
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Blood
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- By the author/editor
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Xochelli, Aliki
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Kalpadakis, Chri ...
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Gardiner, Anne
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Baliakas, Panagi ...
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Vassilakopoulos, ...
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Mould, Sarah
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show more...
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Davis, Zadie
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Stalika, Evangel ...
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Kanellis, George
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Angelopoulou, Ma ...
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McIver-Brown, Ne ...
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Ibbotson, Rachel
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Sachanas, Sotiri ...
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Korkolopoulou, P ...
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Athanasiadou, An ...
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Anagnostopoulos, ...
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Papadaki, Helen ...
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Papadaki, Theodo ...
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Stamatopoulos, K ...
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Pangalis, Gerass ...
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Oscier, David
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- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Hematology
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Blood
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Uppsala University