Division of Hematology, Department of Internal Medicine, Mayo Clinic Rochester, Rochester, MN.
Blood Adv. 2018 Apr 10;2(7):769-776. doi: 10.1182/bloodadvances.2018016782.
We evaluated the impact of light chain type, lambda (λ) or kappa (κ), on disease features and outcomes in patients with immunoglobulin light chain (AL) amyloidosis receiving stem cell transplant at the Mayo Clinic between October 2002 and August 2016. Patients with λ AL amyloidosis had higher rates of renal and neurological involvement (λ 69% vs κ 57%, = .02 and λ 16% vs κ 9%, = .03, respectively). Patients with κ AL amyloidosis had more hepatic involvement (λ 7% vs κ 18%, = .0003). Complete response rate was 43% for both groups and overall response rates were similar (λ 85% vs κ 91%, = .12). Patients with κ light chain amyloidosis had better progression-free and overall survival (PFS: λ 74 months vs κ 101 months, = .0064 and OS: λ 121 months vs κ not reached, = .003). Mayo stage 2004 was more predictive of survival in the λ cohort (median OS of 143 months stage I vs 77 months stage II vs 33 months stage III, < .0001) than in the κ cohort (median OS not reached for stage I and II and 102 months for stage III, = .044). Conditioning dose predicted survival in the λ cohort only (median OS 149 months for melphalan 200 mg/m vs 50 months for melphalan <200 mg/m, < .0001; median OS κ not reached for melphalan 200 mg/m or <200 mg/m, = .38). On multivariate analysis, light chain type remained an independent predictor of survival. Light chain type predicts organ involvement and survival in patients with AL amyloidosis receiving stem cell transplant.
我们评估了轻链类型(λ或κ)对 2002 年 10 月至 2016 年 8 月在梅奥诊所接受干细胞移植的免疫球蛋白轻链(AL)淀粉样变性患者的疾病特征和结局的影响。λ型 AL 淀粉样变性患者的肾脏和神经受累发生率较高(λ为 69%,κ为 57%, =.02;λ为 16%,κ为 9%, =.03)。κ型 AL 淀粉样变性患者肝脏受累更为常见(λ为 7%,κ为 18%, =.0003)。两组完全缓解率分别为 43%,总缓解率相似(λ为 85%,κ为 91%, =.12)。κ轻链淀粉样变性患者的无进展生存期和总生存期更好(PFS:λ为 74 个月,κ为 101 个月, =.0064;OS:λ为 121 个月,κ未达到, =.003)。梅奥 2004 分期在 λ 队列中对生存的预测更具预测性(I 期的中位 OS 为 143 个月,II 期为 77 个月,III 期为 33 个月, <.0001),而在 κ 队列中(I 期和 II 期的中位 OS 未达到,III 期为 102 个月, =.044)。仅在 λ 队列中,预处理剂量预测生存(中位 OS 为 149 个月的马法兰 200 mg/m 与马法兰 <200 mg/m 的 50 个月, <.0001;马法兰 200 mg/m 或 <200 mg/m 的 κ 中位 OS 未达到, =.38)。多变量分析显示,轻链类型仍是生存的独立预测因素。轻链类型可预测接受干细胞移植的 AL 淀粉样变性患者的器官受累和生存情况。