Division of Hematology, Mayo Clinic, Rochester, MN, USA.
Department of Laboratory Medicine and Pathology, Division of Hematopathology, Mayo Clinic, Rochester, MN, USA.
Amyloid. 2020 Mar;27(1):13-16. doi: 10.1080/13506129.2019.1666709. Epub 2019 Sep 23.
Response assessment in light chain (AL) amyloidosis is challenging given the low level of circulating free light chains usually seen. Multi-parametric flow cytometry (MFC) from a marrow aspirate was demonstrated to retain a prognostic significance in several recent studies. In this work, 82 AL patients who had MFC study at end of therapy were analysed based on whether clonal plasma cells were detected or not. Among patients who achieved deep response (i.e. very good partial response or complete response) to first-line therapy, lack of clonal marrow plasma cells as measured by MFC was associated with improved progression-free survival (PFS) compared to patients with residual clonal plasma cells (3-year PFS 88% vs. 46%, = .003), particularly among patients who achieved a complete response (3-year PFS 100% vs. 33%, = .001). Absence of clonal plasma cells by MFC compared with patients with detectable clonal plasma cells among deep responders was associated with lower level of involved light chain (involved free light chain (iFLC), median 1.1 vs. 1.7 mg/dL; = .02) and higher frequency of renal response (100% vs. 68%; = .005). Further studies are needed to determine if MFC should be incorporated into response criteria in AL amyloidosis.
由于通常观察到循环游离轻链水平较低,因此轻链 (AL) 淀粉样变性的反应评估具有挑战性。来自骨髓抽吸物的多参数流式细胞术 (MFC) 在最近的几项研究中被证明保留了预后意义。在这项工作中,根据是否检测到克隆浆细胞,对 82 名在治疗结束时进行 MFC 研究的 AL 患者进行了分析。在接受一线治疗达到深度缓解(即非常好的部分缓解或完全缓解)的患者中,与仍有克隆浆细胞的患者相比,MFC 测量的骨髓浆细胞缺乏克隆性与改善无进展生存期 (PFS) 相关(3 年 PFS 为 88% vs. 46%,=0.003),特别是在完全缓解的患者中(3 年 PFS 为 100% vs. 33%,=0.001)。与深度缓解者中可检测到克隆浆细胞的患者相比,MFC 检测到的克隆浆细胞缺乏与受累轻链水平较低(受累游离轻链 (iFLC),中位数为 1.1 与 1.7mg/dL;=0.02)和更高的肾脏反应频率(100%与 68%;=0.005)相关。需要进一步的研究来确定 MFC 是否应纳入 AL 淀粉样变性的反应标准。