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Flow cytometry-based immunophenotypic analysis of primary systemic light chain amyloidosis

机译:基于流式细胞仪的原发性系统性轻链淀粉样变性的免疫表型分析

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摘要

Using flow cytometry, the present study aimed to investigate the immunophenotypic characteristics of malignant plasma cells (PCs) in the bone marrow of patients with primary systemic light chain amyloidosis (AL) compared with the characteristics of patients with multiple myeloma (MM). Flow cytometric results of 51 patients with AL and 150 patients with MM were reviewed. The proportion of total bone marrow PCs in the patients with AL was significantly lower than that in the patients with MM, 1.35% (0.3–9.5%) vs. 9.7% (0.4–75.7%); (P<0.001). The cells in 24/51 patients with AL comprised two distinct populations: Normal PCs and malignant PCs, whereas only 11/150 patients with MM exhibited two populations. Patients with AL exhibited an increased cluster of differentiation (CD)19 expression compared with patients with MM, 4.5% (0.1–80.9%) vs. 1.6% (0.1–33.6%; P<0.001) and reduced CD138, with 90.8% (30.4–99.9%) vs. 95.0% (40.7–100%; P=0.006) and CD56, with 61.0% (1.5–99.2%) vs. 98.3% (0.1–100%; P<0.001) expression in malignant PCs. Light chain restriction was identified in all patients with AL, and the proportion of λ light chain restriction was 72.5%. The immunophenotypic characteristics of patients with AL demonstrated by flow cytometry were different compared with the characteristics of patients with MM in the number and composition of plasma cells, and the intracellular and extracellular expression of antigens. These differences may be associated with a less malignant phenotype of clonal PCs in AL than MM.
机译:使用流式细胞仪,本研究旨在调查原发性系统性轻链淀粉样变性(AL)患者骨髓中恶性浆细胞(PCs)与多发性骨髓瘤(MM)患者特征的免疫表型特征。回顾了51例AL患者和150例MM患者的流式细胞仪结果。 AL患者的总骨髓PCs比例明显低于MM患者,分别为1.35%(0.3–9.5%)和9.7%(0.4–75.7%); (P <0.001)。 24/51例AL患者的细胞包括两个不同的群体:正常PC和恶性PC,而只有11/150 MM的患者表现出两个群体。与MM患者相比,AL患者表现出分化簇(CD)19表达增加,分别为4.5%(0.1–80.9%)和1.6%(0.1–33.6%; P <0.001),而CD138降低,为90.8%(分别为30.4–99.9%)和95.0%(40.7–100%; P = 0.006)和CD56,其中61.0%(1.5–99.2%)与98.3%(0.1–100%; P <0.001)在恶性PC中表达。在所有AL患者中均鉴定出轻链限制,λ轻链限制的比例为72.5%。流式细胞术证实的AL患者的免疫表型特征与MM患者的特征在于浆细胞的数量和组成以及抗原在细胞内和细胞外的表达。这些差异可能与AL中的MM较PC的恶性表型有关。

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