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Blood, 15 March 2002, Vol. 99, No. 6, pp. 2262-2264

BRIEF REPORT

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

Gerard Tobin, Ulf Thunberg, Anna Johnson, Ingrid Thörn, Ola Söderberg, Magnus Hultdin, Johan Botling, Gunilla Enblad, Jan Sällström, Christer Sundström, Göran Roos, and Richard Rosenquist

From the Departments of Genetics and Pathology and Oncology, Radiology and Clinical Immunology, Uppsala University, Uppsala, Sweden, and the Department of Medical Biosciences, Pathology, Umeå University, Umeå, Sweden.

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 lambda  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.

© 2002 by The American Society of Hematology.
 

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