Somatically mutated Ig VH3-21 genes characterize a new subset of chronic lymphocytic leukemia

G Tobin, U Thunberg, A Johnson… - Blood, The Journal …, 2002 - ashpublications.org
G Tobin, U Thunberg, A Johnson, I Thörn, O Söderberg, M Hultdin, J Botling, G Enblad…
Blood, The Journal of the American Society of Hematology, 2002ashpublications.org
Recent studies on the immunoglobulin variable heavy chain (IgVH) genes have revealed
that B-cell chronic lymphocytic leukemia (B-CLL) consists of at least 2 clinical entities with
either somatically mutated or unmutated VH genes. We have analyzed the VH gene
mutation status and VH gene usage in 119 B-CLL cases and correlated them to overall
survival. A novel finding was the preferential use of the VH3-21 gene in mutated cases,
whereas biased VH1-69 gene usage was found in unmutated cases as previously reported …
Abstract
Recent studies on the immunoglobulin variable heavy chain (IgVH) genes have revealed that B-cell chronic lymphocytic leukemia (B-CLL) consists of at least 2 clinical entities with either somatically mutated or unmutated VH genes. We have analyzed the VH gene mutation status and VH gene usage in 119 B-CLL cases and correlated them to overall survival. A novel finding was the preferential use of the VH3-21 gene in mutated cases, whereas biased VH1-69 gene usage was found in unmutated cases as previously reported. Interestingly, the subset of mutated cases using the VH3-21 gene displayed distinctive genotypic/phenotypic characteristics with shorter average length of the complementarity determining region 3 and clonal expression of λ light chains. In addition, this mutated subset showed significantly shorter survival than other mutated cases and a similar clinical course to unmutated cases. We therefore suggest that B-CLL cases with mutated VH3-21 genes may constitute an additional entity of B-CLL.
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