Chronic Lymphocytic Leukemia With t(2;14)(p16;q32) Involves the BCL11A and IgH Genes and Is Associated With Atypical Morphologic Features and Unmutated IgVH Genes | Zendy

C. Cameron Yin | Zendy; Katherine I. Lin | Zendy; Rhett P. Ketterling | Zendy; Ryan A. Knudson | Zendy; L. Jeffrey Medeiros | Zendy; Lynn L. Barron | Zendy; Yang O. Huh | Zendy; Rajyalakshmi Luthra | Zendy; Michael J. Keating | Zendy; Lynne V. Abruzzo | Zendy

Open Access

Chronic Lymphocytic Leukemia With t(2;14)(p16;q32) Involves the BCL11A and IgH Genes and Is Associated With Atypical Morphologic Features and Unmutated IgVH Genes

Author(s) -

C. Cameron Yin,

Katherine I. Lin,

Rhett P. Ketterling,

Ryan A. Knudson,

L. Jeffrey Medeiros,

Lynn L. Barron,

Yang O. Huh,

Rajyalakshmi Luthra,

Michael J. Keating,

Lynne V. Abruzzo

Publication year - 2009

Publication title -

american journal of clinical pathology

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 0.859

H-Index - 128

eISSN - 1943-7722

pISSN - 0002-9173

DOI - 10.1309/ajcpxly46upflisc

Subject(s) - immunophenotyping , lymphocytosis , chronic lymphocytic leukemia , biology , hepatosplenomegaly , gene rearrangement , pathology , karyotype , fluorescence in situ hybridization , leukemia , cytogenetics , microbiology and biotechnology , immunology , gene , flow cytometry , genetics , medicine , chromosome , disease

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/IgH 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/IgH rearrangement is characterized by atypical morphologic features and unmutated IgV(H) genes.

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