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在诊断时和一线治疗结束时,多参数流式细胞术在 AL 淀粉样变性中的预后价值。

The prognostic value of multiparametric flow cytometry in AL amyloidosis at diagnosis and at the end of first-line treatment.

机构信息

Division of Hematology.

Division of Hematopathology, Department of Laboratory Medicine and Pathology, and.

出版信息

Blood. 2017 Jan 5;129(1):82-87. doi: 10.1182/blood-2016-06-721878. Epub 2016 Oct 11.

Abstract

Multiparametric flow cytometry (MFC) in amyloid light-chain (AL) amyloidosis has not been widely adopted and, consequently, there is little information on its clinical relevance. We studied 173 patients with AL amyloidosis who underwent MFC immunophenotyping of bone marrow sample at diagnosis and 82 patients at the end of the first line of treatment (EOT). The number of monotypic plasma cells (PCs) and the polytypic PCs/bone marrow PCs (pPCs/BMPCs) ratio were analyzed. At diagnosis, ≥2.5% monotypic PCs was associated with a shorter progression-free survival (PFS) and overall survival (OS) compared with patients with <2.5% monotypic PCs (2-year PFS 41% vs 56%, P = .007; 2-year OS 55% vs 70%; P = .01). Additionally, patients with a pPCs/BMPCs ratio of ≤5% had a shorter PFS compared with patients with pPCs/BMPCs ratio >5% (2-year PFS 43% vs 55%; P = .02), but without OS difference (2-year OS 60% vs 67%; P = .19). In a multivariate analysis, the monotypic PCs retained an independent prediction for PFS/OS, whereas the pPCs/BMPCs ratio retained significance only for PFS. At EOT, ≥0.1% monotypic PCs was associated with a shorter PFS and OS compared with patients with <0.1% monotypic PCs (2-year PFS 31% vs 87%; P < .0001; 2-year OS 87% vs 98%, P = .02). In a subgroup analysis among patients who attained a very good partial response or better, the monotypic PCs at the 0.1% cutoff was predictive for progression rate but not for PFS/OS. MFC is prognostic for AL amyloidosis at diagnosis and at EOT. MFC may have a role in the definition of hematologic response.

摘要

多参数流式细胞术(MFC)在轻链淀粉样变性(AL)中的应用尚未广泛普及,因此,其临床相关性的相关信息有限。我们研究了 173 例在诊断时接受骨髓样本 MFC 免疫表型分析的 AL 淀粉样变性患者和 82 例在一线治疗结束时(EOT)的患者。分析了单克隆浆细胞(PCs)的数量和多克隆 PCs/骨髓 PCs(pPCs/BMPCs)的比值。在诊断时,与 <2.5%单克隆 PCs 的患者相比,≥2.5%单克隆 PCs 与较短的无进展生存期(PFS)和总生存期(OS)相关(2 年 PFS 分别为 41%和 56%,P =.007;2 年 OS 分别为 55%和 70%,P =.01)。此外,pPCs/BMPCs 比值≤5%的患者与 pPCs/BMPCs 比值 >5%的患者相比,PFS 更短(2 年 PFS 分别为 43%和 55%,P =.02),但 OS 无差异(2 年 OS 分别为 60%和 67%,P =.19)。在多变量分析中,单克隆 PCs 对 PFS/OS 具有独立的预测作用,而 pPCs/BMPCs 比值仅对 PFS 有意义。在 EOT 时,与 <0.1%单克隆 PCs 的患者相比,≥0.1%单克隆 PCs 与更短的 PFS 和 OS 相关(2 年 PFS 分别为 31%和 87%,P <.0001;2 年 OS 分别为 87%和 98%,P =.02)。在达到非常好的部分缓解或更好的患者亚组分析中,0.1%截点处的单克隆 PCs 可预测进展率,但不能预测 PFS/OS。MFC 在诊断时和 EOT 时对 AL 淀粉样变性具有预后意义。MFC 可能在血液学反应的定义中具有作用。

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