Wong K F, Chan J K, Sin V C
Department of Pathology, Queen Elizabeth Hospital, Kowloon, Hong Kong, China.
Cancer Genet Cytogenet. 1999 Jun;111(2):149-51. doi: 10.1016/s0165-4608(98)00236-2.
T-cell prolymphocytic leukemia (T-PLL) is an uncommon chronic lymphoproliferative disorder characterized by lymphadenopathy, splenomegaly, and lymphocytosis. The leukemic cells have the appearance of prolymphocytes and usually an immunophenotype of T-helper cells (CD3+ CD4+ CD8-). Inv(14q), del(11q), i(8q), and rearranged Xq28 are the commonest nonrandom chromosomal abnormalities in T-PLL. Recently, it has been shown that the ataxia-telangiectasia 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. We report a case of T-PLL with t(6;11)(q21;q23) as the sole chromosomal abnormality and suggest that the cytogenetically identified translocation also implicates the ATM gene.
T细胞幼淋巴细胞白血病（T-PLL）是一种罕见的慢性淋巴细胞增殖性疾病，其特征为淋巴结病、脾肿大和淋巴细胞增多。白血病细胞具有幼淋巴细胞的外观，通常具有T辅助细胞的免疫表型（CD3+ CD4+ CD8-）。Inv(14q)、del(11q)、i(8q)和重排的Xq28是T-PLL中最常见的非随机染色体异常。最近的研究表明，位于11q23的共济失调毛细血管扩张症突变（ATM）基因在T-PLL中经常缺失，提示ATM基因在T-PLL肿瘤发生中具有肿瘤抑制作用。我们报告1例以t(6;11)(q21;q23)作为唯一染色体异常的T-PLL病例，并提示细胞遗传学鉴定的易位也涉及ATM基因。
