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Mutated V-H genes and preferential V(H)3-21 use define new subsets of mantle cell lymphoma
- Walsh, SH (author)
- Thorselius, M (author)
-
- Johnson, Anna (author)
- Lund University,Lunds universitet,Tumörmikromiljö,Sektion I,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Tumor microenvironment,Section I,Department of Clinical Sciences, Lund,Faculty of Medicine
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- Soderberg, O (author)
- Jerkernan, M (author)
- Bjorck, E (author)
- Eriksson, I (author)
- Thunberg, U (author)
- Landgren, O (author)
- Lofvenberg, E (author)
- Wallman, K (author)
- Enblad, G (author)
- Sander, B (author)
- Porwit-MacDonald, A (author)
- Sundstrom, C (author)
- Rosenquist, R (author)
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- (creator_code:org_t)
- 2003-03-13
- 2003
- English.
- In: Blood. - : American Society of Hematology. - 1528-0020 .- 0006-4971. ; 101:10, s. 4047-4054
- Journal article (peer-reviewed)
Abstract
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- Mantle cell lymphoma (MCL) is believed to originate from a naive B cell. However, we recently demonstrated that a subset of MCL displayed mutated V-H genes. We also reported restricted use of certain V-H genes. To assess the prognostic impact of these new findings, we performed V-H gene analysis of 110 patients, revealing that 18 (16%) patients had mutated and 92 (84%) patients had unmutated V-H genes. Because the mutation rate was low in the mutated group (2.2%-6.7%), further investigation of the germline V-H gene in T cells from 5 patients with mutated V-H genes was carried out; results showed that the unrearranged V-H gene was identical to the published sequence. These data confirm that the base pair substitutions within the rearranged V-H genes represent hyper-mutations, and indicate germinal center exposure. However, V-H gene mutation status did not correlate with prognosis because there was no difference in clinical outcome between the unmutated and mutated groups. The most frequently used V-H genes were V(H)3-21 (21 patients) and V(H)4-34 (19 patients). A novel finding was that V(H)3-21(+) MCL almost exclusively ex-pressed X light chains and displayed highly restricted use of the V(lambda)3-19 gene. V(H)3-21(+) patients had longer median survival than the remaining patients (53 vs 34 months; P = .03), but they tended to be younger at diagnosis. The combined use Of V(H)3-21/V(lambda)3-19 suggests a possible role for antigen(s) in the pathogenesis of these tumors and indicates that V(H)3-21(+) patients constitute a new MCL entity. (C) 2003 by The American Society of Hematology.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)
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- By the author/editor
- Walsh, SH
- Thorselius, M
- Johnson, Anna
- Soderberg, O
- Jerkernan, M
- Bjorck, E
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- Eriksson, I
- Thunberg, U
- Landgren, O
- Ehinger, Mats
- Lofvenberg, E
- Wallman, K
- Enblad, G
- Sander, B
- Porwit-MacDonald ...
- Dictor, Michael
- Olofsson, Tor
- Sundstrom, C
- Roos, Gunnel
- Rosenquist, R
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- About the subject
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- MEDICAL AND HEALTH SCIENCES
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- Blood
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- Lund University
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