RT Journal Article
T1 Diagnostic screening of paroxysmal nocturnal hemoglobinuria: Prospective multicentric evaluation of the current medical indications.
A1 Morado, Marta
A1 Freire Sandes, Alex
A1 Colado, Enrique
A1 Subirá, Dolores
A1 Isusi, Paloma
A1 Soledad Noya, María
A1 Belén Vidriales, María
A1 Sempere, Amparo
A1 Ángel Díaz, José
A1 Minguela, Alfredo
A1 Álvarez, Beatriz
A1 Serrano, Cristina
A1 Caballero, Teresa
A1 Rey, Mercedes
A1 Pérez Corral, Ana
A1 Cristina Fernández Jiménez, María
A1 Magro, Elena
A1 Lemes, Angelina
A1 Benavente, Celina
A1 Bañas, Helena
A1 Merino, Juana
A1 Castejon, Celine
A1 Gutierrez, Olivier
A1 Rabasa, Pilar
A1 Vescosi Gonçalves, Matheus
A1 Perez-Andres, Martin
A1 Orfao, Alberto
A1 PNH working group of the Iberian Society of Cytometry (SIC), 
K1 flow cytometry
K1 fluorescence cytometry
K1 hematology
K1 myelodysplastic syndrome
K1 standardization
AB Although consensus guidelines have been proposed in 2010 for the diagnostic screening of paroxysmal nocturnal hemoglobinuria (PNH) by flow cytometry (FCM), so far no study has investigated the efficiency of such medical indications in multicentric vs. reference laboratory settings. Here we evaluate the efficiency of consensus medical indications for PNH testing in 3,938 peripheral blood samples submitted to FCM testing in 24 laboratories in Spain and one reference center in Brazil. Overall, diagnostic screening based on consensus medical indications was highly efficient (14% of PNH+ samples) both in the multicenter setting in Spain (10%) and the reference laboratory in Brazil (16%). The highest frequency of PNH+ cases was observed among patients screened because of bone marrow (BM) failure syndrome (33%), particularly among those with aplastic anemia (AA; 45%) and to a less extent also a myelodysplastic syndrome (MDS; 10%). Among the other individuals studied, the most efficient medical indications for PNH screening included: hemolytic anemia (19%), hemoglobinuria (48%) and unexplained cytopenias (9%). In contrast, only a minor fraction of the patients who had been submitted for PNH testing because of unexplained thrombosis in the absence of cytopenia, were positive (0.4%). In summary, our results demonstrate that the current medical indications for PNH screening by FCM are highly efficient, although improved screening algorithms are needed for patients presenting with thrombosis and normal blood cell counts. © 2016 International Clinical Cytometry Society.
YR 2016
FD 2016-10-01
LK http://hdl.handle.net/10668/10420
UL http://hdl.handle.net/10668/10420
LA en
DS RISalud
RD Jul 30, 2025