Hematology Unit, IRCCS Istituto Tumori "Giovanni Paolo II", Bari, Italy.
Oncology Institute of Southern Switzerland (IOSI), Bellinzona, Switzerland.
Hematol Oncol. 2018 Oct;36(4):617-623. doi: 10.1002/hon.2508. Epub 2018 Mar 30.
Follicular lymphoma (FL) generally has an indolent clinical course, but in some patients, a histological transformation (HT) into aggressive entities may take place and often lead to a poorer survival. The rituximab era has seen an improved outcome of FL, including those with HT. The current treatment strategies for transformed FL are based on immunochemotherapy for the cases with HT at the time of diagnosis or as the first event after watchful waiting. Patients transforming after prior treatment of FL usually benefit from autologous stem cell transplant. Unfortunately, early assessment of the transformation risk remains elusive. Recent studies delved the mechanisms of HT, showing that this is a complex process, resulting from a number of epigenetic and genetic lesions occurring in the tumour cell population as well as progressive changes in the tumour microenvironment. This novel knowledge has prompted clinical investigations on a variety of new therapeutic strategies.
滤泡性淋巴瘤（FL）通常具有惰性的临床病程，但在一些患者中，可能会发生组织学转化（HT）为侵袭性实体瘤，这通常会导致更差的生存。利妥昔单抗时代见证了 FL 的预后改善，包括那些伴有 HT 的患者。目前转化型 FL 的治疗策略基于在诊断时或在观察等待后首次发生 HT 的情况下进行免疫化疗。在先前治疗 FL 后发生转化的患者通常受益于自体干细胞移植。不幸的是，早期评估转化风险仍然难以捉摸。最近的研究深入探讨了 HT 的机制，表明这是一个复杂的过程，是肿瘤细胞群体中发生的许多表观遗传和遗传损伤以及肿瘤微环境的渐进性变化共同作用的结果。这些新知识促使对各种新的治疗策略进行了临床研究。
