From the Department of Pathology, Oregon Health & Science University, Portland;
Department of Hematology-Oncology, Knight Cancer Institute, Oregon Health & Science University, Portland;
Am J Clin Pathol. 2014 Sep;142(3):347-54. doi: 10.1309/AJCPUBHM8U7ZFLOB.
B-cell prolymphocytic leukemia (B-PLL) remains a controversial entity, and its molecular pathogenesis is largely unknown. Patients are older, typically having marked lymphocytosis and splenomegaly in the absence of lymphadenopathy. It is defined as a mature B-cell leukemia with more than 55% circulating prolymphocytes. Leukemic mantle cell lymphoma and chronic lymphocytic leukemia in prolymphocytic transformation must be excluded.
Case archives were retrospectively reviewed for B-PLL in patients without a previous diagnosis of chronic lymphocytic leukemia or other B-cell neoplasm.
We identified six cases of B-PLL with available cytogenetic data, five of which showed evidence of aberrations in MYC. Three cases showed additional signals for the MYC gene by fluorescence in situ hybridization (FISH), and two cases demonstrated t(8;14)MYC/IGH by karyotyping or FISH. High levels of MYC protein expression were detected in all cases tested with MYC aberrations.
These results suggest that deregulation of MYC plays an important role in the pathogenesis of B-PLL and expands the spectrum of B-cell neoplasms associated with aberrations of MYC.
B 细胞前淋巴细胞白血病（B-PLL）仍然是一个有争议的实体，其分子发病机制在很大程度上尚不清楚。患者年龄较大，通常有明显的淋巴细胞增多和脾肿大，而无淋巴结病。它被定义为一种成熟的 B 细胞白血病，有超过 55%的循环前淋巴细胞。必须排除白血病套细胞淋巴瘤和慢性淋巴细胞白血病前淋巴细胞转化。
对无慢性淋巴细胞白血病或其他 B 细胞肿瘤既往诊断的患者的 B-PLL 病例档案进行回顾性审查。
我们确定了 6 例有可用细胞遗传学数据的 B-PLL，其中 5 例显示 MYC 异常。3 例通过荧光原位杂交（FISH）显示 MYC 基因的额外信号，2 例通过核型分析或 FISH 显示 t(8;14)MYC/IGH。所有经 MYC 异常检测的病例均检测到高水平的 MYC 蛋白表达。
这些结果表明，MYC 的失调在 B-PLL 的发病机制中起着重要作用，并扩展了与 MYC 异常相关的 B 细胞肿瘤谱。
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