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

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dc.authorid0000-0002-3385-8359
dc.contributor.authorGören Şahin, Deniz
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.authorAkgün Çağlıyan, Gülsüm
dc.contributor.authorAksu, Salih
dc.contributor.authorDoğu, Mehmet Hilmi
dc.contributor.authorDal, Mehmet Sinan
dc.contributor.authorKarakuş, Volkan
dc.contributor.authorGemici, Aliihsan
dc.contributor.authorTerzi, Hatice
dc.contributor.authorKelkitli, Engin
dc.contributor.authorŞıvgın, 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.accessioned2021-10-11T07:43:32Z
dc.date.available2021-10-11T07:43:32Z
dc.date.issued2021
dc.departmentİstanbul Medipol Üniversitesi, Tıp Fakültesi, Dahili Tıp Bilimleri Bölümü, İç Hastalıkları Ana Bilim Dalı
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.
dc.identifier.citationGören Şahin, D., Akay, O. M., Keklik, M., Okan, V., Karakuş, A., Demir, C. ... Altuntaş, F. (2021). Clinical characteristics and therapeutic outcomes of paroxysmal nocturnal hemoglobinuria patients in Turkey: A multicenter experience. Annals of Hematology, 100(7), 1667-1675. https://dx.doi.org/10.1007/s00277-021-04554-4
dc.identifier.doi10.1007/s00277-021-04554-4
dc.identifier.endpage1675
dc.identifier.issn0939-5555
dc.identifier.issn1432-0584
dc.identifier.issue7
dc.identifier.scopusqualityQ1
dc.identifier.startpage1667
dc.identifier.urihttps://dx.doi.org/10.1007/s00277-021-04554-4
dc.identifier.urihttps://hdl.handle.net/20.500.12511/8409
dc.identifier.volume100
dc.identifier.wosqualityQ2
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakScopus
dc.indekslendigikaynakPubMed
dc.language.isoen
dc.publisherSpringer
dc.relation.ispartofAnnals of Hematologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanı
dc.rightsinfo:eu-repo/semantics/embargoedAccess
dc.subjectEculizumab
dc.subjectHemolysis
dc.subjectParoxysmal Nocturnal Hemoglobinuria
dc.subjectThrombosis
dc.titleClinical characteristics and therapeutic outcomes of paroxysmal nocturnal hemoglobinuria patients in Turkey: A multicenter experience
dc.typeArticle

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