Division of Endocrinology, Diabetes and Metabolism, Hematology and Rheumatology, Second Department of Internal Medicine, Graduate School of Medicine, University of the Ryukyus, Nishihara, Japan.
Clinical Research Support Office, National Cancer Center Hospital East, Kashiwa, Japan.
Cancer Sci. 2018 Jul;109(7):2286-2293. doi: 10.1111/cas.13641. Epub 2018 Jun 16.
Aggressive adult T-cell leukemia/lymphoma (ATL) has an extremely poor prognosis and is hyperendemic in Okinawa, Japan. This study evaluated two prognostic indices (PIs) for aggressive ATL, the ATL-PI and Japan Clinical Oncology Group (JCOG)-PI, in a cohort from Okinawa. The PIs were originally developed using two different Japanese cohorts that included few patients from Okinawa. The endpoint was overall survival (OS). Multivariable Cox regression analyses in the cohort of 433 patients revealed that all seven factors for calculating each PI were statistically significant prognostic predictors. Three-year OS rates for ATL-PI were 35.9% (low-risk, n = 66), 10.4% (intermediate-risk, n = 256), and 1.6% (high-risk, n = 111), and those for JCOG-PI were 22.4% (moderate-risk, n = 176) and 5.3% (high-risk, n = 257). The JCOG-PI moderate-risk group included both the ATL-PI low- and intermediate-risk groups. ATL-PI more clearly identified the low-risk patient subgroup than JCOG-PI. To evaluate the external validity of the two PIs, we also assessed prognostic discriminability among 159 patients who loosely met the eligibility criteria of a previous clinical trial. Three-year OS rates for ATL-PI were 34.5% (low-risk, n = 42), 9.2% (intermediate-risk, n = 109), and 12.5% (high-risk, n = 8). Those for JCOG-PI were 22.4% (moderate-risk, n = 95) and 7.6% (high-risk, n = 64). The low-risk ATL-PI group had a better prognosis than the JCOG-PI moderate-risk group, suggesting that ATL-PI would be more useful than JCOG-PI for establishing and examining novel treatment strategies for ATL patients with a better prognosis. In addition, strongyloidiasis, previously suggested to be associated with ATL-related deaths in Okinawa, was not a prognostic factor in this study.
侵袭性成人 T 细胞白血病/淋巴瘤（ATL）预后极差，在日本冲绳地区高发。本研究评估了两种侵袭性 ATL 预后指数（PI），即 ATL-PI 和日本临床肿瘤学组（JCOG）-PI，在冲绳队列中的应用。这些 PI 最初是使用两个不同的日本队列开发的，其中包括来自冲绳的少数患者。终点是总生存期（OS）。对 433 例患者的多变量 Cox 回归分析显示，每个 PI 计算的七个因素均为统计学上显著的预后预测因子。ATL-PI 的 3 年 OS 率分别为低危组（n=66）35.9%、中危组（n=256）10.4%和高危组（n=111）1.6%，JCOG-PI 的 3 年 OS 率分别为中危组（n=176）22.4%和高危组（n=257）5.3%。JCOG-PI 中的中危组包括 ATL-PI 的低危和中危组。ATL-PI 比 JCOG-PI 更能明确低危患者亚组。为了评估两个 PI 的外部有效性，我们还评估了 159 例患者的预后判别能力，这些患者大致符合先前临床试验的入选标准。ATL-PI 的 3 年 OS 率分别为低危组（n=42）34.5%、中危组（n=109）9.2%和高危组（n=8）12.5%。JCOG-PI 的 3 年 OS 率分别为中危组（n=95）22.4%和高危组（n=64）7.6%。低危 ATL-PI 组的预后优于 JCOG-PI 中危组，提示 ATL-PI 对于建立和检验预后较好的 ATL 患者新的治疗策略可能比 JCOG-PI 更有用。此外，本研究中，先前提示与冲绳地区 ATL 相关死亡相关的寄生虫感染（Strongyloidiasis）不是预后因素。
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