Clinical Haematology, Peter MacCallum Cancer Centre and The Royal Melbourne Hospital, Melbourne, VIC, Australia; Blood Cells and Blood Cancer Division, Walter and Eliza Hall Institute of Medical Research, Parkville, VIC, Australia; Faculty of Medicine, Dentistry and Health Sciences, University of Melbourne, Melbourne, VIC, Australia.
Clinical Haematology, Peter MacCallum Cancer Centre and The Royal Melbourne Hospital, Melbourne, VIC, Australia; Faculty of Medicine, Dentistry and Health Sciences, University of Melbourne, Melbourne, VIC, Australia.
Lancet Haematol. 2023 Feb;10(2):e142-e154. doi: 10.1016/S2352-3026(22)00355-6.
Mantle cell lymphoma is an uncommon subtype of lymphoma characterised by clinical and biological heterogeneity. Although most patients with mantle cell lymphoma have durable responses after chemoimmunotherapy, there is a need to prospectively identify high-risk subsets of patients for whom disease control with standard chemotherapy will be short lived. Among the available prognostic factors, TP53 mutations are uniquely informative owing to their strong association with early disease progression and death among patients receiving conventional chemoimmunotherapy, with the highest negative prognostic value compared with other established risk indicators, including the mantle cell lymphoma international prognostic index, histological features, elevated Ki-67, and other genetic lesions. The poor outcomes for patients with TP53-mutated mantle cell lymphoma receiving chemoimmunotherapy and second-line Bruton tyrosine kinase inhibitors represent an urgent need for alternative approaches. In this Review, we synthesise the available data to inform the management of this high-risk subset of patients and present a treatment strategy prioritising clinical trials and early use of cellular therapies.
套细胞淋巴瘤是一种罕见的淋巴瘤亚型，其特征为临床和生物学异质性。尽管大多数套细胞淋巴瘤患者在接受化疗免疫治疗后有持久缓解，但仍需要前瞻性地识别高危亚组患者，这些患者接受标准化疗的疾病控制时间将很短。在现有的预后因素中，TP53 突变是唯一具有信息性的，因为它们与接受常规化疗免疫治疗的患者早期疾病进展和死亡密切相关，与其他已确立的风险指标相比，其预后价值最高，包括套细胞淋巴瘤国际预后指数、组织学特征、Ki-67 升高和其他遗传病变。TP53 突变的套细胞淋巴瘤患者接受化疗免疫治疗和二线布鲁顿酪氨酸激酶抑制剂治疗的不良预后，这迫切需要替代方法。在这篇综述中，我们综合了现有数据，为这一高危亚组患者的管理提供信息，并提出了一种治疗策略，优先考虑临床试验和早期使用细胞疗法。
