Management of patients with relapsed or refractory (R/R) AL amyloidosis is complex. Some initial reports have shown positive results with daratumumab in heavily pre-treated AL amyloidosis patients. In this retrospective multicentric study, 38 patients (mean age 64 ± 9 years) with R/R AL amyloidosis treated with daratumumab were included. Cardiac and renal involvement was present in 76 and 74% of patients, and 42% had ≥3 organs involved. Median number of previous lines of therapy was 2 (range 1–8). Overall hematological response was 72%, including 28% complete responses. The median time to first hematological response was 2 weeks. A high-quality response (≥very good partial response) was obtained in 65% of patients who had never achieved such depth of response previously. Hematological responses were more frequent among patients receiving daratumumab as second-line therapy compared to subsequent therapies (92 vs. 61%). Cardiac and renal organ response rates were 37 and 59%. At 12 months, overall and progression-free survival were 59% (95%CI: 0.36–0.77) and 52% (95%CI: 0.29–0.70), respectively. Daratumumab is a safe and effective drug in the treatment of R/R AL amyloidosis and should be considered early in the course of the disease.

患有复发或难治性（R / R）AL淀粉样变性的患者的治疗很复杂。一些初步报告显示，在严重预处理的AL淀粉样变性患者中，daratumumab的阳性结果。在这项回顾性多中心研究中，纳入了38例（平均年龄64±9岁）患有daratumumab治疗的R / R AL淀粉样变性的患者。76％和74％的患者存在心脏和肾脏受累，其中≥3个器官受累的患者占42％。先前治疗的中位数为2（范围1-8）。总体血液学反应为72％，包括28％的完全反应。首次血液学反应的中位时间为2周。在以前从未达到过这种反应深度的患者中，有65％的患者获得了高质量的反应（≥很好的部分反应）。与随后的治疗相比，接受达拉妥单抗作为二线治疗的患者血液学反应更为频繁（92比61％）。心脏和肾脏器官反应率分别为37％和59％。在12个月时，总生存期和无进展生存期分别为59％（95％CI：0.36-0.77）和52％（95％CI：0.29-0.70）。Daratumumab是治疗R / R AL淀粉样变性的安全有效药物，应在病程早期考虑。