Puerta, J.E. (José Enrique) de la

Filtering

20191

Settings

Author search.filters.isAuthorOfPublication.75ae7bef-4444-48b7-8e90-58f00b4fe07a

Search Results

Now showing 1 - 1 of 1
  • Thumbnail Image
    Flow cytometry for fast screening and automated risk assessment in systemic light-chain amyloidosis
    (Nature, 2019) González, M.E. (María Esther); Cedena, M.T. (María Teresa); Verde, J. (Javier); Pérez, J.J. (José J.); Martínez-López, J. (Joaquín); Krsnik, I. (Isabel); Gironella, M. (Mercedes); Orfao, A. (Alberto); Vidriales, M.B. (María Belén); Ocio, E.M. (Enrique M.); Mateos, M.V. (María Victoria); Arriba, F. (Felipe) de; Puerta, J.E. (José Enrique) de la; Puig, N. (Noemí); Labrador, J. (Jorge); Burgos, L. (Leire); Lasa, M. (Marta); Palomera, L. (Luis); Lahuerta, J.J. (Juan José); Pérez-Montaña, A. (Albert); Gómez-Toboso, D. (Dolores); Paiva, B. (Bruno); Lecumberri, R. (Ramón); Oriol, A. (Albert); Rubia, J. (Javier) de la; Prosper-Cardoso, F. (Felipe); Casanova, M. (María); Lecrevisse, Q. (Quentin); Merino, J. (Juana); San-Miguel, J.F. (Jesús F.); Moreno, C. (Cristina); Cabañas, V. (Valentín); García-de-Coca, A. (Alfonso)
    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.