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

Autor: Goren Sahin D; Department of Hematology, Demiroglu Bilim University, Istanbul, Turkey. drdenizgoren@gmail.com., Akay OM; Department of Hematology, Koc University, Istanbul, Turkey., Keklik M; Department of Hematology, Erciyes University, Kayseri, Turkey., Okan V; Department of Hematology, Gaziantep University, Gaziantep, Turkey., Karakus A; Department of Hematology, Dicle University, Diyarbakir, Turkey., Demir C; Gazi Yasargil Training and Research Hospital, University of Health Sciences, Diyarbakir, Turkey., Erkurt MA; Department of Hematology, Inonu University, Malatya, Turkey., Ilkkilic K; Recep Tayyip Erdogan University Training and Research Hospital, Rize, Turkey., Yildirim R; Division of Hematology, Medical Park Antalya Hospital, Antalya, Turkey., Akgun Cagliyan G; Department of Hematology, Pamukkale University, Denizli, Turkey., Aksu S; Department of Hematology, Hacettepe University, Ankara, Turkey., Dogu MH; Istanbul Training and Research Hospital, University of Health Sciences, Istanbul, Turkey., Dal MS; Ankara Oncology Training and Research Hospital, University of Health Sciences, Ankara, Turkey., Karakus V; Mugla Sitki Kocman University Training and Research Hospital, Mugla, Turkey., Gemici AI; Department of Hematology, Istanbul Medipol University, Istanbul, Turkey., Terzi H; Department of Hematology, Cumhuriyet University, Sivas, Turkey., Kelkitli E; Department of Hematology, Ondokuz Mayis University, Samsun, Turkey., Sivgin S; Division of Hematology, Acibadem Kayseri Hospital, Kayseri, Turkey., Unal A; Department of Hematology, Erciyes University, Kayseri, Turkey., Yilmaz M; Department of Hematology, Gaziantep University, Gaziantep, Turkey., Ayyildiz O; Department of Hematology, Dicle University, Diyarbakir, Turkey., Korkmaz S; Kayseri City Training and Research Hospital, University of Health Sciences, Kayseri, Turkey., Eser B; Division of Hematology, Medical Park Antalya Hospital, Antalya, Turkey., Altuntas F; Ankara Oncology Training and Research Hospital, University of Health Sciences, Ankara, Turkey.; Department of Hematology, Yildirim Beyazit University, Ankara, Turkey.
Jazyk: angličtina
Zdroj: Annals of hematology [Ann Hematol] 2021 Jul; Vol. 100 (7), pp. 1667-1675. Date of Electronic Publication: 2021 May 14.
DOI: 10.1007/s00277-021-04554-4
Abstrakt: The 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.
Databáze: MEDLINE
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