Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
Semin Hematol. 2011 Jul;48(3):155-65. doi: 10.1053/j.seminhematol.2011.04.001.
Mantle cell lymphoma (MCL) is a B-cell neoplasia genetically characterized by the t(11;14)(q13;q32) translocation leading to the overexpression of its target gene CCND1. The aggressive clinical behavior of this tumor has been considered to be influenced by its genetic and molecular pathogenesis that integrates an accumulation of many chromosomal aberrations associated with frequent alterations in cell cycle and DNA damage response mechanisms and activation of cell survival pathways. Recent studies aimed to define new chromosomal regions, target genes, and signaling pathways that may contribute to the pathogenesis of this tumor. A subset of patients presenting with a leukemic and non-nodal disease and following a more indolent clinical evolution seem to have some differences in their chromosomal and genomic profiles compared to patients with conventional MCL. The new studies are opening new perspectives on the pathogenesis of this lymphoma that may influence our clinical practice in the diagnosis and management of patients.
套细胞淋巴瘤（MCL）是一种 B 细胞肿瘤，其遗传特征为 t（11；14）（q13；q32）易位，导致其靶基因 CCND1 的过度表达。这种肿瘤侵袭性的临床行为被认为受到其遗传和分子发病机制的影响，其中包括许多染色体异常的积累，这些异常与细胞周期和 DNA 损伤反应机制的频繁改变以及细胞存活途径的激活有关。最近的研究旨在确定可能有助于该肿瘤发病机制的新染色体区域、靶基因和信号通路。与传统 MCL 患者相比，一部分表现为白血病和非淋巴结疾病且临床演变更为惰性的患者，其染色体和基因组图谱似乎存在一些差异。这些新的研究为这种淋巴瘤的发病机制开辟了新的视角，可能会影响我们在诊断和治疗患者方面的临床实践。
