Division of Hematology, Mayo Clinic, Rochester, MN, USA.
Divsion of Nephrology and Hypertension, Mayo Clinic, Rochester, MN, USA.
Leukemia. 2019 May;33(5):1268-1272. doi: 10.1038/s41375-019-0400-5. Epub 2019 Feb 8.
It is well known that staging of patients with AL amyloidosis at diagnosis predicts for survival, but there is a paucity of literature delineating the prognostic value of these systems at relapse. We evaluated the prognostic value of AL staging among 413 patients initiated with second-line therapy between 2000 and 2015. Both the Revised Mayo 2012 and the European revision of Mayo 2004 staging systems were used. The median time from initial treatment to second-line therapy was 11.7 months. The first-line therapy was autologous stem cell transplant (ASCT) in 179 (43%) patients and non-ASCT therapies in 234 patients. Median survival from the institution of second-line therapy was 61 months. Both the Mayo 2004 and 2012 staging systems were of prognostic benefit at second-line therapy with respective risk ratios of 2.78 (95% CI: 2.15, 3.58) and 3.03 (95% CI: 2.33, 3.93) for patients with > stage 2 disease. On multivariate analysis, the predictive value of staging at second-line therapy was independent of stage at diagnosis and prior ASCT as first-line therapy. This study indicates that the Mayo staging systems work well at second-line therapy. Consequently, it is suitable for the stratification of patients in trials for relapsed, refractory AL amyloidosis.
众所周知，在诊断时对淀粉样变性病患者进行分期可预测生存情况，但有关这些系统在复发时的预后价值的文献却很少。我们评估了在 2000 年至 2015 年间接受二线治疗的 413 例患者中，AL 分期的预后价值。使用了修订后的 2012 年 Mayo 分期系统和欧洲修订的 2004 年 Mayo 分期系统。从初始治疗到二线治疗的中位时间为 11.7 个月。一线治疗为自体干细胞移植（ASCT）的患者有 179 例（43%），而非 ASCT 治疗的患者有 234 例。从二线治疗开始的中位生存时间为 61 个月。在二线治疗中，2004 年和 2012 年 Mayo 分期系统均具有预后获益，相应的风险比分别为 2.78（95%CI：2.15，3.58）和 3.03（95%CI：2.33，3.93），适用于疾病分期>2 的患者。多变量分析表明，二线治疗时的分期预测价值独立于诊断时的分期和作为一线治疗的 ASCT。本研究表明 Mayo 分期系统在二线治疗中效果良好。因此，它适合用于复发、难治性淀粉样变性病患者临床试验的分层。
