Publication:
Study for the diagnostic screening of paroxysmal nocturnal hemoglobinuria in older patients with unexplained anemia and/or cytopenia

dc.contributor.authorOzdemir, Zehra N.
dc.contributor.authorIlhan, Osman
dc.contributor.authorÖzet, Gülsüm
dc.contributor.authorFalay, Mesude
dc.contributor.authorYenerel, Mustafa
dc.contributor.authorTuğlular, Tulin
dc.contributor.authorTurgut, Mehmet
dc.contributor.authorGüvenç, Birol
dc.contributor.authorUnal, Ali
dc.contributor.authorAyyıldız, Orhan
dc.contributor.authorAndıç, Neslihan
dc.contributor.authorHacihanefioğlu, Abdullah
dc.contributor.authorŞahin, Fahri
dc.contributor.authorŞencan, Mehmet
dc.contributor.authorÖzsan, Güner H.
dc.contributor.authorYıldırım, Rahşan
dc.contributor.authorTiftik, Eyüp N.
dc.contributor.authorTombak, Anil
dc.contributor.authorSalim, Ozan
dc.contributor.authorKaya, Emin
dc.contributor.authorAkay, Olga M.
dc.contributor.authorOkan, Vahap
dc.contributor.authorPehlivan, Mustafa
dc.contributor.authorSaydam, Güray
dc.contributor.buuauthorAli, Ridvan
dc.contributor.buuauthorALİ, RIDVAN
dc.contributor.departmentTıp Fakültesi
dc.contributor.departmentHematoloji Ana Bilim Dalı
dc.contributor.researcheridGXD-8209-2022
dc.date.accessioned2024-07-05T13:04:59Z
dc.date.available2024-07-05T13:04:59Z
dc.date.issued2020-01-01
dc.description.abstractBackground: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired hematopoietic stem cell disease that may lead to weakness and death of patients, if unrecognized and untreated. Although consensus guidelines were reviewed recently for the diagnostic screening of PNH with multi-parameter flow cytometry (FCM), until now, no study has investigated the efficiency of such clinical indications in older patients.Methods: Overall, 20 centers participated in the study and a total of 1,689 patients were included, 313 of whom were at geriatric age and 1,376 were aged 18 - 64 years. We evaluated the efficiency of consensus clinical indications for PNH testing using FCM in peripheral blood samples and compared the results of older patients and patients aged 18 - 64 years.Results: PNH clones were detected positive in 7/313 (2.2%) of the older patients. Five (74.4%) of the patients with PNH clones had aplastic anemia, 1 had unexplained cytopenia, and 1 patient had myelodysplastic syndrome (MDS) with refractory anemia. PNH clones were not detected in any older patients who were screened for unexplained thrombosis, Coombs (-) hemolytic anemia, hemoglobinuria, and others (e.g., elevated lactate dehydrogenase (LDH), splenomegaly). We detected PNH clones in 55/1376 (4%) samples of the patients aged under 65 years. Forty-two (76.4%) patients with PNH clones had aplastic anemia, 2 patients had Coombs (-) hemolytic anemia, 3 patients had unexplained cytopenia, 1 patient had MDS with refractory anemia, 1 patient had hemoglobinuria, and 6 (10.9%) had others (e.g., elevated LDH, splenomegaly). PNH clones were not detected in any patients who were screened for unexplained thrombosis. There was no statistical difference between the geriatric population and patients aged 18 - 64 years in terms of clinical indications for PNH screening with FCM (p = 0.49).Conclusions: Our results showed that the current clinical indications for PNH screening with FCM were also efficient in older patients. We suggest that older patients with unexplained anemia, myelodysplastic syndrome with refractory anemia, and unexplained cytopenia should be screened for PNH with FCM to identify patients who would benefit from treatment.
dc.identifier.doi10.7754/Clin.Lab.2020.191218
dc.identifier.endpage1715
dc.identifier.issn1433-6510
dc.identifier.issue9
dc.identifier.startpage1709
dc.identifier.urihttps://doi.org/10.7754/Clin.Lab.2020.191218
dc.identifier.urihttps://hdl.handle.net/11452/43006
dc.identifier.volume66
dc.identifier.wos000581034500007
dc.indexed.wosWOS.SCI
dc.language.isoen
dc.publisherClin Lab Publ
dc.relation.journalClinical Laboratory
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi
dc.rightsinfo:eu-repo/semantics/closedAccess
dc.subjectPig-a mutations
dc.subjectMyelodysplastic syndromes
dc.subjectPnh
dc.subjectGuidelines
dc.subjectDisorders
dc.subjectClones
dc.subjectAdults
dc.subjectParoxysmal nocturnal hemoglobinuria
dc.subjectOlder patients
dc.subjectMultiparameter flow cytometry
dc.subjectConsensus based clinical indications
dc.subjectScience & technology
dc.subjectLife sciences & biomedicine
dc.subjectMedical laboratory technology
dc.titleStudy for the diagnostic screening of paroxysmal nocturnal hemoglobinuria in older patients with unexplained anemia and/or cytopenia
dc.typeArticle
dspace.entity.typePublication
local.contributor.departmentTıp Fakültesi/Hematoloji Ana Bilim Dalı
relation.isAuthorOfPublication578cac50-ce4d-4818-8e99-2634ef340753
relation.isAuthorOfPublication.latestForDiscovery578cac50-ce4d-4818-8e99-2634ef340753

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