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Diagnostic screening of paroxysmal nocturnal hemoglobinuria: Prospective multicentric evaluation of the current medical indications
Author(s) -
Morado Marta,
Freire Sandes Alex,
Colado Enrique,
Subirá Dolores,
Isusi Paloma,
Soledad Noya María,
Belén Vidriales María,
Sempere Amparo,
Ángel Díaz José,
Minguela Alfredo,
Álvarez Beatriz,
Serrano Cristina,
Caballero Teresa,
Rey Mercedes,
Pérez Corral Ana,
Cristina Fernández Jiménez María,
Magro Elena,
Lemes Angelina,
Benavente Celina,
Bañas Helena,
Merino Juana,
Castejon Celine,
Gutierrez Olivier,
Rabasa Pilar,
Vescosi Gonçalves Matheus,
PerezAndres Martin,
Orfao Alberto
Publication year - 2017
Publication title -
cytometry part b: clinical cytometry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.646
H-Index - 61
eISSN - 1552-4957
pISSN - 1552-4949
DOI - 10.1002/cyto.b.21480
Subject(s) - paroxysmal nocturnal hemoglobinuria , medicine , hemoglobinuria , aplastic anemia , cytopenia , bone marrow failure , myelodysplastic syndromes , pediatrics , anemia , bone marrow , stem cell , genetics , haematopoiesis , biology
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