TY - JOUR
T1 - 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
AU - Yin, C. Cameron
AU - Lin, Katherine I.Chun
AU - Ketterling, Rhett P.
AU - Knudson, Ryan A.
AU - Medeiros, L. Jeffrey
AU - Barron, Lynn L.
AU - Huh, Yang O.
AU - Luthra, Rajyalakshmi
AU - Keating, Michael J.
AU - Abruzzo, Lynne V.
PY - 2009/5
Y1 - 2009/5
N2 - 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.
AB - 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.
KW - Atypical morphologic features
KW - BCL11A
KW - Chronic lymphocytic leukemia/small lymphocytic lymphoma
KW - IgV mutation
KW - T(2;14)(p16;q32)
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U2 - 10.1309/AJCPXLY46UPFLISC
DO - 10.1309/AJCPXLY46UPFLISC
M3 - Article
C2 - 19369625
AN - SCOPUS:65349131189
SN - 0002-9173
VL - 131
SP - 663
EP - 670
JO - American journal of clinical pathology
JF - American journal of clinical pathology
IS - 5
ER -