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Flow cytometry for fast screening and automated risk assessment in systemic light-chain amyloidosis

2018; Springer Nature; Volume: 33; Issue: 5 Linguagem: Inglês

10.1038/s41375-018-0308-5

1476-5551

Noemí Puig, Bruno Paiva, Marta Lasa, Leire Burgos, José J. Pérez, Juana Merino, Cristina Moreno, María‐Belén Vidriales, Dolores Gómez Toboso, María‐Teresa Cedena, Enrique M. Ocio, Ramón Lecumberri, Alfonso García de Coca, Jorge Labrador, Esther González-García, Luis Palomera, Mercedes Gironella, Valentín Cabañas, María Casanova, Albert Oriol, Isabel Krsnik, Albert Pérez-Montaña, Javier de la Rubia, Jose-Enrique de la Puerta, Felipe de Arriba, Felipe Prósper, Joaquin Martínez‐López, Quentin Lécrevisse, Javier Verde, María‐Victoria Mateos, Juan José Lahuerta, Alberto Órfão, Jesús F. San Miguel,

Chronic Lymphocytic Leukemia Research

Early diagnosis and risk stratification are key to improve outcomes in light-chain (AL) amyloidosis. Here we used multidimensional-flow-cytometry (MFC) to characterize bone marrow (BM) plasma cells (PCs) from a series of 166 patients including newly-diagnosed AL amyloidosis (N = 94), MGUS (N = 20) and multiple myeloma (MM, N = 52) vs. healthy adults (N = 30). MFC detected clonality in virtually all AL amyloidosis (99%) patients. Furthermore, we developed an automated risk-stratification system based on BMPCs features, with independent prognostic impact on progression-free and overall survival of AL amyloidosis patients (hazard ratio: ≥ 2.9;P ≤ .03). Simultaneous assessment of the clonal PCs immunophenotypic protein expression profile and the BM cellular composition, mapped AL amyloidosis in the crossroad between MGUS and MM; however, lack of homogenously-positive CD56 expression, reduction of B-cell precursors and a predominantly-clonal PC compartment in the absence of an MM-like tumor PC expansion, emerged as hallmarks of AL amyloidosis (ROC-AUC = 0.74;P < .001), and might potentially be used as biomarkers for the identification of MGUS and MM patients, who are candidates for monitoring pre-symptomatic organ damage related to AL amyloidosis. Altogether, this study addressed the need for consensus on how to use flow cytometry in AL amyloidosis, and proposes a standardized MFC-based automated risk classification ready for implementation in clinical practice.