Janssen Oncology, Toronto, ON, Canada.
Department of Lymphoma/Myeloma, The University of MD Anderson Cancer Center, Houston, TX, 77030, USA.
Ann Hematol. 2019 Jan;98(1):175-183. doi: 10.1007/s00277-018-3501-8. Epub 2018 Oct 13.
Follicular lymphoma (FL) and marginal zone lymphoma (MZL) are two subtypes of indolent B cell non-Hodgkin lymphoma (NHL) that account for approximately 20% and 12% of all NHLs, respectively. FL and MZL are rare conditions with orphan disease designations. We conducted a comprehensive review of the burden of FL and MZL that encompasses the epidemiological, real world clinical, economic, and humanistic impact of these diseases globally. A targeted literature search identified 31 eligible studies for review. Epidemiological coverage was poor, with data obtained for studies from only seven countries. The incidences of both subtypes were low: age-standardized incidence rates of FL ranged from 2.1/100,000 in France to 4.3/100,000 in the USA, while for MZL it varied geographically from 0.5/100,000 in Australia to 2.6/100,000 in the UK. The cumulative total direct healthcare costs for FL were higher for patients with progressive disease compared to those without ($30,890 vs. $8704 at 12 months, respectively) and main driver of costs related to the use of chemotherapy. Five-year overall survival was improved in patients with FL compared with MZL (e.g., 76.5% vs 60.7% in one study that reported on both subtypes). Mortality rates were particularly lower in female patients with FL aged < 60 years. However, limited outcome data for MZL patients were identified. FL and MZL contribute significant burden on healthcare systems and on patients globally, with delays in progression potentially leading to cost savings. More rigorous characterization of these two NHL subtypes, new and more effective treatments, and standardization of reporting would lead to a more robust understanding of future data in this disease area.
滤泡性淋巴瘤(FL)和边缘区淋巴瘤(MZL)是两种惰性 B 细胞非霍奇金淋巴瘤(NHL)亚型,分别占所有 NHL 的 20%和 12%左右。FL 和 MZL 是罕见疾病,具有孤儿病的指定。我们对 FL 和 MZL 的负担进行了全面审查,包括这些疾病在全球的流行病学、真实世界临床、经济和人文影响。进行了针对性的文献检索,以审查 31 项合格研究。流行病学报道不足,仅从七个国家的研究中获得数据。两种亚型的发病率均较低:FL 的年龄标准化发病率范围从法国的 2.1/100,000 到美国的 4.3/100,000,而 MZL 的发病率在地理上从澳大利亚的 0.5/100,000 到英国的 2.6/100,000 不等。与无进展疾病患者相比,进展性疾病患者的 FL 累积直接医疗保健费用更高(分别为 30,890 美元和 8,704 美元,在 12 个月时),并且是与化疗使用相关的成本的主要驱动因素。与 MZL 相比,FL 患者的五年总生存率得到改善(例如,在一项同时报告两种亚型的研究中,分别为 76.5%和 60.7%)。在年龄<60 岁的女性 FL 患者中,死亡率尤其较低。但是,确定了有限的 MZL 患者的结果数据。FL 和 MZL 对全球的医疗保健系统和患者造成了重大负担,进展延迟可能会节省成本。更严格地描述这两种 NHL 亚型、新的和更有效的治疗方法以及报告的标准化将导致对该疾病领域未来数据的更深入了解。