Chronic lymphocytic leukemia with t(2;14)(p16;q32) involves the BCL11A and IgH genes and is associated With atypical morphologic features and unmutated IgV H genes

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Abstract

The t(2;14)(p16;q32) has been reported previously in only 12 cases of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). The clinicopathologic features have been incompletely described. We describe 6 new cases of CLL/SLL with t(2;14)(p16;q32). All had marrow involvement, 4 had absolute lymphocytosis, 4 had lymphadenopathy, and 3 had hepatosplenomegaly. All showed atypical lymphocyte morphologic features with plasmacytoid differentiation and irregular nuclei; 3 had increased prolymphocytes. Flow cytometry demonstrated typical immunophenotypes in 5 and an atypical immunophenotype in 1. All expressed ZAP70; 5 assessed showed unmutated IgV H genes. Karyotyping identified t(2;14)(p16;q32) as the sole abnormality in 1, primary abnormality in 2, and part of a complex karyotype in 3. Fluorescence in situ hybridization analysis revealed BCL11A/lgH rearrangement in all. After chemotherapy, 3 patients died of disease and 3 were alive with disease (median follow-up, 80 months). We conclude that CLL/SLL with t(2;14) (p16;q32) and BCL11A/lgH rearrangement is characterized by atypical morphologic features and unmutated IgV H genes. © American Society for Clinical Pathology.

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Yin, C. C., Lin, K. I. C., Ketterling, R. P., Knudson, R. A., Medeiros, L. J., Barron, L. L., … Abruzzo, L. V. (2009). Chronic lymphocytic leukemia with t(2;14)(p16;q32) involves the BCL11A and IgH genes and is associated With atypical morphologic features and unmutated IgV H genes. American Journal of Clinical Pathology, 131(5), 663–670. https://doi.org/10.1309/AJCPXLY46UPFLISC

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