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Cytometry B Clin Cytom. 2017 Sep;92(5):361-370. doi: 10.1002/cyto.b.21480. Epub 2016 Oct 1.

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

Author information

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

Abstract

BACKGROUND:

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.

METHODS:

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.

RESULTS:

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%).

CONCLUSIONS:

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.

KEYWORDS:

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

PMID:
27598686
DOI:
10.1002/cyto.b.21480
[Indexed for MEDLINE]
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