T-cell prolymphocytic leukemia with a novel translocation (6;11)(q21;q23)

T-cell prolymphocytic leukemia (T-PLL) is an uncommon chronic lymphoprolife rative disorder characterized by lymphadenopathy, splenomegaly, and lymphoc ytosis. The leukemic cells have the appearance of prolymphocytes and usuall y an immunophenotype of T-helper cells (CD3+ CD4+ CD8-). Inv(14q), del(11q) , i(8q), and rearranged Xq28 are the commonest nonrandom chromosomal abnorm alities in T-PLL. Recently, it has been shown that the ataxia-telangiectasi a mutated (ATM) gene located at 11q23 is often deleted in T-PLL, suggesting a tumor suppressor role of the ATM gene on tumorigenesis of T-PLL. Mie rep ort a case of T-PLL with t(6;11)(q21;q23) as the sole chromosomal abnormali ty and suggest that the cytogenetically identified translocation also impli cates the ATM gene. (C) Elsevier Science, Inc., 1999. All rights reserved.