Diagnostic screening of paroxysmal nocturnal hemoglobinuria: Prospective multicentric evaluation of the current medical indications.
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.
Servicio Hematologia. Hospital Universitario La Paz, Madrid, Spain.
Marta
M
Morado
División de Hematología y Citometría de Flujo, Fleury Group, São Paulo, Brazil.
Alex
A
Freire Sandes
Servicio Hematologia. Hospital Central Asturias, Oviedo, Spain.
Enrique
E
Colado
Servicio Hematologia. Hospital Universitario Guadalajara, Guadalajara, Spain.
Dolores
D
Subirá
Servicio Hematologia. Hospital Basurto, Bilbao, Spain.
Paloma
P
Isusi
Servicio Hematologia. Complejo Hospitalario Universitario A Coruña, A Coruña, Spain.
María
M
Soledad Noya
Department of Hematology, Hospital Universitario de Salamanca, Instituto de Investigación Biomédica de Salamanca (IBSAL), Spain; Instituto de Biología Molecular y Celular del Cáncer (CIC-CSIC), Salamanca, Spain.
María
M
Belén Vidriales
Servicio Hematologia. Hospital Universitario La Fe, Valencia, Spain.
Amparo
A
Sempere
Servicio Hematologia. Hospital Universitario Santiago de Compostela, Santiago de Compostela, Spain.
José
J
Ángel Díaz
Servicio de Inmunologia. Hospital Virgen de la Arrixaca e Instituto Murciano de Investigación Biosanitaria (IMIB), Murcia, Spain.
Alfredo
A
Minguela
Laboratorio Central Comunidad de Madrid, Madrid, Spain.
Beatriz
B
Álvarez
Servicio Hematologia. Fundacion Jimenez Diaz, Madrid, Spain.
Cristina
C
Serrano
Servicio Hematologia. Hospital Universitario Virgen del Rocio, Sevilla, Spain.
Teresa
T
Caballero
Laboratorio de Inmunologia, Hospital Donostia, San Sebastián, Spain.
Mercedes
M
Rey
Hospital Gregorio Marañon, Madrid, Spain.
Ana
A
Pérez Corral
Servicio Hematologia. Complejo Hospitalario de Toledo, Toledo, Spain.
María
M
Cristina Fernández Jiménez
Servicio Hematologia. Hospital Universitario Principe de Asturias, Alcalá de Henares, Spain.
Elena
E
Magro
Servicio Hematologia. Hospital Universitario Dr. Negrin. Las Palmas de Gran Canaria, Spain.
Angelina
A
Lemes
Servicio Hematologia. Hospital Clínico San Carlos, Madrid, Spain.
Celina
C
Benavente
Servicio Hematologia. Hospital San Pedro Alcantara, Caceres, Spain.
Helena
H
Bañas
Servicio Inmunologia. Clinica Universitaria de Navarra, Spain.
Juana
J
Merino
Balague Center. de Llobregat, Spain.
Celine
C
Castejon
Servicio Hematologia. Hospital Rio Hortega, Valladolid, Spain.
Olivier
O
Gutierrez
Servicio Hematologia. Hospital San Pedro, Logroño, Spain.
Pilar
P
Rabasa
División de Hematología y Citometría de Flujo, Fleury Group, São Paulo, Brazil.
Matheus
M
Vescosi Gonçalves
Cancer Research Centre (IBMCC, USAL-CSIC), Institute for Biomedical Research of Salamanca (IBSAL) and Department of Medicine and Cytometry Service (NUCLEUS Research Support Platform), University of Salamanca (USAL), Salamanca, Spain.
Martin
M
Perez-Andres
Cancer Research Centre (IBMCC, USAL-CSIC), Institute for Biomedical Research of Salamanca (IBSAL) and Department of Medicine and Cytometry Service (NUCLEUS Research Support Platform), University of Salamanca (USAL), Salamanca, Spain.
Alberto
A
Orfao
D000741:Anemia, Aplastic / Q000175:diagnosis* / Q000453:epidemiology / Q000378:metabolism; D004912:Erythrocytes / Q000166:cytology*; D005260:Female; D005434:Flow Cytometry / Q000379:methods; D006457:Hemoglobinuria, Paroxysmal / Q000175:diagnosis* / Q000453:epidemiology* / Q000378:metabolism; D006801:Humans; D008297:Male; D009190:Myelodysplastic Syndromes / Q000175:diagnosis* / Q000453:epidemiology / Q000378:metabolism; D015995:Prevalence; D011446:Prospective Studies; D012189:Retrospective Studies
flow cytometry; fluorescence cytometry; hematology; myelodysplastic syndrome; standardization