دورية
Flow cytometry for fast screening and automated risk assessment in systemic light-chain amyloidosis
العنوان: | Flow cytometry for fast screening and automated risk assessment in systemic light-chain amyloidosis |
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المؤلفون: | Puig, Noemi, Paiva, Bruno, Lasa, Marta, Burgos, Leire, Perez, Jose J., Merino, Juana, Moreno, Cristina, Vidriales, Maria-Belen, Toboso, Dolores Gómez, Cedena, Maria-Teresa, Ocio, Enrique M., Lecumberri, Ramon, García de Coca, Alfonso, Labrador, Jorge, Gonzalez, Maria-Esther, Palomera, Luis, Gironella, Mercedes, Cabañas, Valentin, Casanova, Maria, Oriol, Albert, Krsnik, Isabel, Pérez-Montaña, Albert, de la Rubia, Javier, de la Puerta, Jose-Enrique, de Arriba, Felipe, Prosper, Felipe, Martinez-Lopez, Joaquin, Lecrevisse, Quentin, Verde, Javier, Mateos, Maria-Victoria, Lahuerta, Juan-Jose, Orfao, Alberto, San Miguel, Jesús F. |
المصدر: | Leukemia; May 2019, Vol. 33 Issue: 5 p1256-1267, 12p |
مستخلص: | 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. |
قاعدة البيانات: | Supplemental Index |
تدمد: | 08876924 14765551 |
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DOI: | 10.1038/s41375-018-0308-5 |