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Cytometry B Clin Cytom. 2016 Sep 6. doi: 10.1002/cyto.b.21480. [Epub ahead of print]

Diagnostic screening of paroxysmal nocturnal hemoglobinuria: Prospective multicentric evaluation of the current medical indications.

Author information

  • 1Servicio Hematologia. Hospital Universitario La Paz, Madrid, Spain.
  • 2División de Hematología y Citometría de Flujo, Fleury Group, São Paulo, Brazil.
  • 3Servicio Hematologia. Hospital Central Asturias, Oviedo, Spain.
  • 4Servicio Hematologia. Hospital Universitario Guadalajara, Guadalajara, Spain.
  • 5Servicio Hematologia. Hospital Basurto, Bilbao, Spain.
  • 6Servicio Hematologia. Complejo Hospitalario Universitario A Coruña, A Coruña, Spain.
  • 7Department 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.
  • 8Servicio Hematologia. Hospital Universitario La Fe, Valencia, Spain.
  • 9Servicio Hematologia. Hospital Universitario Santiago de Compostela, Santiago de Compostela, Spain.
  • 10Servicio de Inmunologia. Hospital Virgen de la Arrixaca e Instituto Murciano de Investigación Biosanitaria (IMIB), Murcia, Spain.
  • 11Laboratorio Central Comunidad de Madrid, Madrid, Spain.
  • 12Servicio Hematologia. Fundacion Jimenez Diaz, Madrid, Spain.
  • 13Servicio Hematologia. Hospital Universitario Virgen del Rocio, Sevilla, Spain.
  • 14Laboratorio de Inmunologia, Hospital Donostia, San Sebastián, Spain.
  • 15Hospital Gregorio Marañon, Madrid, Spain.
  • 16Servicio Hematologia. Complejo Hospitalario de Toledo, Toledo, Spain.
  • 17Servicio Hematologia. Hospital Universitario Principe de Asturias, Alcalá de Henares, Spain.
  • 18Servicio Hematologia. Hospital Universitario Dr. Negrin. Las Palmas de Gran Canaria, Spain.
  • 19Servicio Hematologia. Hospital Clínico San Carlos, Madrid, Spain.
  • 20Servicio Hematologia. Hospital San Pedro Alcantara, Caceres, Spain.
  • 21Servicio Inmunologia. Clinica Universitaria de Navarra, Spain.
  • 22Balague Center. de Llobregat, Spain.
  • 23Servicio Hematologia. Hospital Rio Hortega, Valladolid, Spain.
  • 24Servicio Hematologia. Hospital San Pedro, Logroño, Spain.
  • 25Cancer 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.
  • 26Cancer 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.



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.


flow cytometry; fluorescence cytometry; hematology; myelodysplastic syndrome; standardization

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