Department of Medicine III, LMU Hospital, Munich, Germany.
Hematol Oncol. 2021 Jun;39 Suppl 1:31-38. doi: 10.1002/hon.2860.
Mantle cell lymphoma (MCL) is clinically characterized by its heterogenous behavior with courses ranging from indolent cases that do not require therapy for years to highly aggressive MCL with very limited prognosis. A better understanding of the complex biology of MCL has already led to the approval of several innovative agents, expanding the landscape of MCL therapies and improving therapeutic options especially for refractory or relapsed disease. Nevertheless, to further optimize MCL treatment, early identification of individual risk profile and risk-adapted, patient-tailored choice of therapeutic strategy needs to be prospectively incorporated in clinical patient management. This review highlights recent advances in deciphering the molecular background of MCL, the definition of prognostically relevant factors and the identification of potential druggable targets and summarizes current treatment recommendations for primary and relapsed/refractory MCL including novel targeted therapies.
套细胞淋巴瘤(MCL)在临床上表现为异质性,病程从多年无需治疗的惰性病例到预后极差的侵袭性 MCL 不等。对 MCL 复杂生物学的更好理解已经导致了几种创新药物的批准,扩大了 MCL 治疗的范围,并改善了治疗选择,特别是对于难治性或复发性疾病。然而,为了进一步优化 MCL 的治疗,需要前瞻性地将个体风险特征的早期识别和风险适应性、个体化的治疗策略选择纳入临床患者管理中。本综述强调了阐明 MCL 分子背景、确定具有预后相关性的因素以及确定潜在可用药靶点方面的最新进展,并总结了原发性和复发性/难治性 MCL 的当前治疗建议,包括新型靶向治疗。