Clinical characteristics and therapeutic outcomes of paroxysmal nocturnal hemoglobinuria patients in Turkey: a multicenter experience

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dc.authoridKelkitli, Engin/0000-0001-7342-1760
dc.authoridKeklik, Muzaffer/0000-0002-6426-5249
dc.authoriddemir, cengiz/0000-0001-9856-184X
dc.authoridDogu, Mehmet Hilmi/0000-0001-7237-2637
dc.authoridilkkilic, kadir/0000-0003-1136-0514
dc.authoridokan, vahap/0000-0002-9869-8181
dc.authoridYILMAZ, Mehmet/0000-0002-5710-5263
dc.authorwosidAkgun Cagliyan, Gulsum/AAA-5330-2022
dc.authorwosidKelkitli, Engin/AAC-3607-2019
dc.authorwosidAyyildiz, Orhan/AEO-2377-2022
dc.authorwosidKeklik, Muzaffer/B-3037-2016
dc.authorwosiddemir, cengiz/JZE-3811-2024
dc.authorwosidDogu, Mehmet Hilmi/W-2255-2017
dc.authorwosidilkkilic, kadir/CAF-7643-2022
dc.contributor.authorGoren Sahin, Deniz
dc.contributor.authorAkay, Olga Meltem
dc.contributor.authorKeklik, Muzaffer
dc.contributor.authorOkan, Vahap
dc.contributor.authorKarakus, Abdullah
dc.contributor.authorDemir, Cengiz
dc.contributor.authorErkurt, Mehmet Ali
dc.date.accessioned2024-08-04T20:50:14Z
dc.date.available2024-08-04T20:50:14Z
dc.date.issued2021
dc.departmentİnönü Üniversitesien_US
dc.description.abstractThe aim of this study is to collect paroxysmal nocturnal hemoglobinuria (PNH) patient data from hematology centers all over Turkey in order to identify clinical features and management of PNH patients. Patients with PNH were evaluated by a retrospective review of medical records from 19 different institutions around Turkey. Patient demographics, medical history, laboratory findings, and PNH-specific information, including symptoms at the diagnosis, complications, erythrocyte, and granulocyte clone size, treatment, and causes of death were recorded. Sixty patients (28 males, 32 females) were identified. The median age was 33 (range; 17-77) years. Forty-six patients were diagnosed as classic PNH and 14 as secondary PNH. Fatigue and abdominal pain were the most frequent presenting symptoms. After eculizumab became available in Turkey, most of the patients (n = 31/46, 67.4%) were switched to eculizumab. Three patients with classic PNH underwent stem cell transplantation. The median survival time was 42 (range; 7-183 months) months. This study is the first and most comprehensive review of PNH cases in Turkey. It provided us useful information to find out the differences between our patients and literature, which may help us understand the disease.en_US
dc.identifier.doi10.1007/s00277-021-04554-4
dc.identifier.endpage1675en_US
dc.identifier.issn0939-5555
dc.identifier.issn1432-0584
dc.identifier.issue7en_US
dc.identifier.pmid33988739en_US
dc.identifier.scopus2-s2.0-85105936013en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.startpage1667en_US
dc.identifier.urihttps://doi.org/10.1007/s00277-021-04554-4
dc.identifier.urihttps://hdl.handle.net/11616/99926
dc.identifier.volume100en_US
dc.identifier.wosWOS:000650535000001en_US
dc.identifier.wosqualityQ2en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherSpringeren_US
dc.relation.ispartofAnnals of Hematologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectEculizumaben_US
dc.subjectHemolysisen_US
dc.subjectParoxysmal nocturnal hemoglobinuriaen_US
dc.subjectThrombosisen_US
dc.titleClinical characteristics and therapeutic outcomes of paroxysmal nocturnal hemoglobinuria patients in Turkey: a multicenter experienceen_US
dc.typeArticleen_US

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