Study for the diagnostic screening of paroxsymal nocturnal hemoglobinuria in Turkey: Prospective multicentric evaluation of suspected patients.
Diagnostic screening
Multiparameter flow cytometry
Paroxysmal nocturnal hemoglobinuria
Journal
Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis
ISSN: 1473-0502
Titre abrégé: Transfus Apher Sci
Pays: England
ID NLM: 101095653
Informations de publication
Date de publication:
Oct 2019
Oct 2019
Historique:
received:
22
05
2019
revised:
23
07
2019
accepted:
16
08
2019
pubmed:
23
9
2019
medline:
30
4
2020
entrez:
23
9
2019
Statut:
ppublish
Résumé
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease presenting with variable and various clinical findings. PNH might be overlooked and diagnosis may be delayed due to low awareness about PNH. This is the first multicenter study in Turkey, investigating the efficiency of diagnostic screening of PNH by multiparameter flow cytometry (FCM) according to consensus guidelines. We evaluate the efficiency of consensus clinical indications for PNH testing with FCM in 1689peripheral blood samples from 20 centers between January 2014 and December 2017. Overall, at the 20 centers contributing to this study, PNH clone were detected in 62/1689 samples (3.6%) by FCM test. 75.8% (n = 47) of patients with PNH clone had aplastic anemia, 3.2% (n = 2) had Coombs (-) hemolytic anemia, 6.5% (n = 4) had unexplained cytopenia, 3.2% (n = 2) had MDS with refractory anemia, 1.6% (n = 1) had hemoglobinuria and 9.7% (n = 6) had others (elevated LDH, splenomegaly, etc.). In contrast, we detect no PNH clone test in patients who were screened for unexplained thrombosis. Our study showed that current clinical indications for PNH testing are highly efficient and diagnostic screening of suspected patients for PNH with FCM is recommended. However, advanced screening algorithms are required for patients presenting with unexplained thrombosis and normal complete blood count.
Sections du résumé
BACKGROUND
BACKGROUND
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease presenting with variable and various clinical findings. PNH might be overlooked and diagnosis may be delayed due to low awareness about PNH. This is the first multicenter study in Turkey, investigating the efficiency of diagnostic screening of PNH by multiparameter flow cytometry (FCM) according to consensus guidelines.
METHODS
METHODS
We evaluate the efficiency of consensus clinical indications for PNH testing with FCM in 1689peripheral blood samples from 20 centers between January 2014 and December 2017.
RESULTS
RESULTS
Overall, at the 20 centers contributing to this study, PNH clone were detected in 62/1689 samples (3.6%) by FCM test. 75.8% (n = 47) of patients with PNH clone had aplastic anemia, 3.2% (n = 2) had Coombs (-) hemolytic anemia, 6.5% (n = 4) had unexplained cytopenia, 3.2% (n = 2) had MDS with refractory anemia, 1.6% (n = 1) had hemoglobinuria and 9.7% (n = 6) had others (elevated LDH, splenomegaly, etc.). In contrast, we detect no PNH clone test in patients who were screened for unexplained thrombosis.
CONCLUSIONS
CONCLUSIONS
Our study showed that current clinical indications for PNH testing are highly efficient and diagnostic screening of suspected patients for PNH with FCM is recommended. However, advanced screening algorithms are required for patients presenting with unexplained thrombosis and normal complete blood count.
Identifiants
pubmed: 31542336
pii: S1473-0502(19)30184-3
doi: 10.1016/j.transci.2019.08.021
pii:
doi:
Types de publication
Clinical Trial
Journal Article
Multicenter Study
Langues
eng
Pagination
659-662Informations de copyright
Copyright © 2019 Elsevier Ltd. All rights reserved.