Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
Curr Opin Hematol. 2012 Jul;19(4):299-304. doi: 10.1097/MOH.0b013e328353bbbd.
Mature B-cell lymphomas bearing concurrent chromosomal rearrangement of MYC/8q24 and BCL2/18q21 are associated with an aggressive clinical course and resistance to conventional chemotherapy. This review summarizes the recent literature regarding the clinical and pathologic features of double-hit lymphomas and outlines current questions about the most accurate and inclusive definition of the disease.
Comprehensive evaluation of large series of aggressive mature B-cell neoplasms reveals recurrent chromosomal aberrations in the majority of cases. A subset of these lymphomas harbors multiple rearrangements, including MYC/8q24 in combination with BCL2/18q21 and/or BCL6/3q27, and displays a particularly aggressive clinical course. Recent data suggest that consideration of additional features, such as copy number alteration, quantitative protein expression, and biologic pathway activation may be important in deriving a more accurate definition of double-hit lymphoma. Despite the poor prognosis associated with this subset of lymphomas, there remains no evidence for a risk-adapted treatment strategy and no clinical, pathologic, or genetic factors that predict response to therapy.
Double-hit lymphoma remains an incompletely characterized disease entity. Large, multicenter studies are needed to define relevant clinical, genetic, and pathologic variables and to characterize appropriate risk-adapted treatment strategies.
同时具有 MYC/8q24 和 BCL2/18q21 染色体重排的成熟 B 细胞淋巴瘤具有侵袭性临床病程和对常规化疗的耐药性。这篇综述总结了关于双打击淋巴瘤的临床和病理特征的最新文献，并概述了目前关于该疾病最准确和全面定义的问题。
对大量侵袭性成熟 B 细胞肿瘤的综合评估显示，大多数病例中存在反复的染色体异常。这些淋巴瘤中有一部分存在多个重排，包括 MYC/8q24 与 BCL2/18q21 和/或 BCL6/3q27 结合，并且表现出特别侵袭性的临床病程。最近的数据表明，考虑其他特征，如拷贝数改变、定量蛋白表达和生物学途径激活，可能对得出更准确的双打击淋巴瘤定义很重要。尽管与这组淋巴瘤相关的预后较差，但仍然没有证据表明存在风险适应治疗策略，也没有预测对治疗反应的临床、病理或遗传因素。
双打击淋巴瘤仍然是一个不完全特征化的疾病实体。需要大型多中心研究来定义相关的临床、遗传和病理变量，并描述适当的风险适应治疗策略。
