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首页> 外文期刊>Modern Pathology >The clinical significance of 8q24/MYC rearrangement in chronic lymphocytic leukemia
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The clinical significance of 8q24/MYC rearrangement in chronic lymphocytic leukemia

机译：8q24 / MYC 重排在慢性淋巴细胞白血病中的临床意义

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Chromosome 8q24/MYC rearrangement is associated with Burkitt lymphoma and some aggressive B-cell lymphomas, but is rare in chronic lymphocytic leukemia. We here report a cohort of 20 chronic lymphocytic leukemia patients with 8q24/MYC rearrangement, 3 detected at time of initial diagnosis and 17 acquired after a median interval of 48 months. At the time when 8q24/MYC arrangement was detected, 18 patients had B-symptoms, 17 had lymphadenopathy, and 17 had splenomegaly. Histologically, typical chronic lymphocytic leukemia morphology was seen in six patients, increased prolymphocytes in nine and Richter鈥檚 transformation in five patients. Eighteen patients had karyotypic information available that showed t(8;v) in a complex karyotype in 12 patients and in a non-complex karyotype in 6 patients. Fluorescence in situ hybridization confirmed MYC rearrangement in 17/17 patients. All patients required therapy after 8q24/MYC rearrangement was detected. At last follow-up, five of six patients with a non-complex karyotype were alive after a median of 74 months (10~143 months) from the detection of 8q24/MYC rearrangement. In contrast, 10 of 12 patients with a complex karyotype died with a median survival of 5.5 months. We conclude that 8q24/MYC rearrangement in chronic lymphocytic leukemia is rare and often acquired during the course of disease. If it is presented in a complex karyotype, it is often associated with Richter鈥檚 transformation, refractory to therapy and an aggressive clinical course; on the other hand, if it is present in a non-complex karyotype, patients often respond to risk-adapted therapies and achieve remission.
机译：染色体8q24 / MYC重排与Burkitt淋巴瘤和一些侵袭性B细胞淋巴瘤有关，但在慢性淋巴细胞性白血病中很少见。我们在此报告了一组20名慢性淋巴细胞性白血病患者，这些患者具有8q24 / MYC重排，在初次诊断时发现3例，中位间隔48个月后获得17例。在检测到8q24 / MYC安排时，有18例B症状，17例淋巴结肿大和17例脾肿大。从组织学上看，在6例患者中观察到典型的慢性淋巴细胞性白血病形态，在9例患者中观察到淋巴细胞增多，在5例患者中观察到Richter转化。 18位患者的核型信息可用，其中t（8; v）在12例患者中为复杂核型，在6例患者中为非复杂核型。荧光原位杂交证实17/17患者中MYC重排。在检测到8q24 / MYC重排后，所有需要治疗的患者。在最后一次随访中，从检测到8q24 / MYC重排后，中位数74个月（10〜143个月）后，有6例非复杂核型的患者中有5例还活着。相比之下，复杂核型的12名患者中有10名死亡，中位生存期为5.5个月。我们得出的结论是，慢性淋巴细胞性白血病中的8q24 / MYC重排很少见，并且通常在疾病过程中获得。如果以复杂的核型出现，通常与里氏转化，治疗难治性和侵袭性临床过程有关。另一方面，如果它存在于非复杂的核型中，则患者通常会对适应风险的疗法产生反应并获得缓解。

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