dc.description.abstract | 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. | en_US |
dc.department-temp | [Goren Sahin, Deniz] Demiroglu Bilim Univ, Dept Hematol, Istanbul, Turkey; [Akay, Olga Meltem] Koc Univ, Dept Hematol, Istanbul, Turkey; [Keklik, Muzaffer] Erciyes Univ, Dept Hematol, Kayseri, Turkey; [Okan, Vahap; Yilmaz, Mehmet] Gaziantep Univ, Dept Hematol, Gaziantep, Turkey; [Karakus, Abdullah; Ayyildiz, Orhan] Dicle Univ, Dept Hematol, Diyarbakir, Turkey; [Demir, Cengiz] Univ Hlth Sci, Gazi Yasargil Training & Res Hosp, Diyarbakir, Turkey; [Erkurt, Mehmet Ali] Inonu Univ, Dept Hematol, Malatya, Turkey; [Ilkkilic, Kadir] Recep Tayyip Erdogan Univ, Training & Res Hosp, Rize, Turkey; [Yildirim, Rahsan; Eser, Bulent] Med Pk Antalya Hosp, Div Hematol, Antalya, Turkey; [Akgun Cagliyan, Gulsum] Pamukkale Univ, Dept Hematol, Denizli, Turkey; [Aksu, Salih] Hacettepe Univ, Dept Hematol, Ankara, Turkey; [Dogu, Mehmet Hilmi] Univ Hlth Sci, Istanbul Training & Res Hosp, Istanbul, Turkey; [Dal, Mehmet Sinan] Univ Hlth Sci, Ankara Oncol Training & Res Hosp, Ankara, Turkey; [Karakus, Volkan] Mugla Sitki Kocman Univ, Training & Res Hosp, Mugla, Turkey; [Gemici, Ali Ihsan] Istanbul Medipol Univ, Dept Hematol, Istanbul, Turkey; [Terzi, Hatice] Cumhuriyet Univ, Dept Hematol, Sivas, Turkey; [Kelkitli, Engin] Ondokuz Mayis Univ, Dept Hematol, Samsun, Turkey; [Sivgin, Serdar] Acibadem Kayseri Hosp, Div Hematol, Kayseri, Turkey; [Korkmaz, Serdal] Univ Hlth Sci, Kayseri City Training & Res Hosp, Kayseri, Turkey; [Altuntas, Fevzi] Yildirim Beyazit Univ, Dept Hematol, Ankara, Turkey | en_US |