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

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dc.authorid0000-0003-2090-4392en_US
dc.authorid0000-0001-5673-8408en_US
dc.contributor.authorŞahin, Deniz Gören
dc.contributor.authorAkay, Olga Meltem
dc.contributor.authorKeklik, Muzaffer
dc.contributor.authorOkan, Vahap
dc.contributor.authorKarakuş, Abdullah
dc.contributor.authorDemir, Cengiz
dc.contributor.authorErkurt, Mehmet Ali
dc.contributor.authorİlkkılıç, Kadir
dc.contributor.authorYıldırım, Rahşan
dc.contributor.authorÇağlıyan, Gülsüm Akgün
dc.contributor.authorAksu, Salih
dc.contributor.authorDoğu, Mehmet Hilmi
dc.contributor.authorDal, Mehmet Sinan
dc.contributor.authorKarakuş, Volkan
dc.contributor.authorGemici, Ali İhsan
dc.contributor.authorTerzi, Hatice
dc.contributor.authorKelkitli, Engin
dc.contributor.authorŞıvgn, Serdar
dc.contributor.authorÜnal, Ali
dc.contributor.authorYılmaz, Mehmet
dc.contributor.authorAyyıldız, Orhan
dc.contributor.authorKorkmaz, Serdal
dc.contributor.authorEser, Bülent
dc.contributor.authorAltuntaş, Fevzi
dc.date.accessioned2022-08-22T10:58:20Z
dc.date.available2022-08-22T10:58:20Z
dc.date.issued2021en_US
dc.departmentDicle Üniversitesi, Tıp Fakültesi, Dahili Tıp Bilimleri Bölümü, İç Hastalıklar Ana Bilim Dalıen_US
dc.descriptionWOS:000650535000001
dc.descriptionPMID: 33988739
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.citationŞahin, D.G., Akay, O.M., Keklik, M., Okan, V., Karakuş, A, Demir, C. ve diğerleri. (2021). Clinical characteristics and therapeutic outcomes of paroxysmal nocturnal hemoglobinuria patients in Turkey: a multicenter experience. Annals of Hematology, 100(7), 1667-1675. https://doi. org/10.1007/s00277-021-04554-4en_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.pmid33988739
dc.identifier.scopus2-s2.0-85105936013
dc.identifier.scopusqualityQ2
dc.identifier.startpage1667en_US
dc.identifier.urihttps://link.springer.com/article/10.1007/s00277-021-04554-4
dc.identifier.urihttps://hdl.handle.net/11468/10230
dc.identifier.volume100en_US
dc.identifier.wosWOS:000650535000001
dc.identifier.wosqualityQ2
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakScopus
dc.indekslendigikaynakPubMed
dc.institutionauthorKarakuş, Abdullah
dc.institutionauthorAyyıldız, Orhan
dc.language.isoenen_US
dc.publisherSpringeren_US
dc.relation.ispartofAnnals of Hematology
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_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.titleClinical characteristics and therapeutic outcomes of paroxysmal nocturnal hemoglobinuria patients in Turkey: a multicenter experience
dc.typeArticleen_US

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