Wos İndeksli Yayınlar Koleksiyonu

Permanent URI for this collectionhttps://hdl.handle.net/11727/4807

Browse

Search Results

Now showing 1 - 3 of 3
  • Item
    Evolution of clinical characteristics of patients with paroxysmal nocturnal hemoglobinuria treated with eculizumab in turkey: a multicenter retrospective analysis
    (2021) Karadag, Fatma Keklik; Yenerel, Mustafa Nuri; Yilmaz, Mehmet; Uskudar, Hava; Ozkocaman, Vildan; Tuglular, Tulin Firatli; Erdem, Fuat; Unal, Ali; Ayyildiz, Orhan; Ozet, Gulsum; Comert, Melda; Kaya, Emin; Ayer, Mesut; Salim, Ozan; Guvenc, Birol; Ozdogu, Hakan; Mehtap, Ozgur; Sonmez, Mehmet; Guler, Nil; Hacioglu, Sibel; Aydogdu, Ismet; Bektas, Ozlen; Toprak, Selami Kocak; Kaynar, Lale; Yagci, Munci; Aksu, Salih; Tombak, Anil; Karakus, Volkan; Yavasoglu, Irfan; Onec, Birgul; Ozcan, Mehmet Ali; Undar, Levent; Ali, Ridvan; Ilhan, Osman; Saydam, Guray; Sahin, Fahri; 34322292
    Paroxysmal nocturnal hemoglobinuria (PNH) is a rare X-linked genetic disorder. On the contrary to its name, it is a multisystemic disease and various symptoms other than hemoglobinuria could be occurred. It could be life threatening especially because of thromboembolic events. In the last decade, a terminal complement inhibition with eculizumab approved with promising results for PNH patients. We conducted this study to evaluate the long term experience of eculizumab therapy from Turkey for the first time. Our cohort included 138 patients with PNH treated with eculizumab between January 2008 and December 2018 at 28 centers in Turkey. Laboratory and clinical findings at the time of diagnosis and after eculizumab therapy were recorded retrospectively. The median age was 39 (range 18-84) years and median granulocyte PNH clone size was 74% (range 3.06-99.84%) at the time of diagnosis. PNH with bone marrow failure syndrome was detected in 49 patients and the rest of 89 patients had classical PNH. Overall 45 patients (32.6%) had a history of any prior thrombotic event before eculizumab therapy and only 2 thrombotic events were reported during the study period. Most common symptoms are fatigue (75.3%), hemoglobinuria (18.1%), abdominal pain (15.2%) and dysphagia (7.9%). Although PNH is commonly related with coombs negativity, we detected coombs positivity in 2.17% of patients. Seven months after the therapy, increased hemoglobin level was seen and remarkably improvement of lactate dehydrogenase level during the treatment was occurred. In addition to previous studies, our real life data support that eculizumab is well tolerated with no serious adverse events and improves the PNH related findings.
  • Thumbnail Image
    Item
    sEPCR Levels in Chronic Myeloproliferative Diseases and Their Association with Thromboembolic Events: A Case-Control Study
    (2014) Atalay, Figen; Toprak, Selami Kocak; Koca, Ebru; Karakus, Sena
    Objective: Venous, arterial, and microcirculatory events are frequently encountered in the clinical course of essential thrombocytosis and polycythemia vera. We aimed to investigate the levels of soluble endothelial protein C receptor (sEPCR) in myeloproliferative diseases to see whether there was a difference between the patients with and without history of thromboembolism. Materials and Methods: The study included patients with polycythemia vera (n=12), patients with essential thrombocytosis (n=13), and controls (n=29). In all groups, we measured proteins C and S, antithrombin and sEPCR levels, and plasma concentrations of thrombin-antithrombin complex, prothrombin fragments 1+2, and D-dimer. Results: Comparing the patients with and without history of thromboembolic attack, statistically significant differences were not observed in terms of sEPCR, D-dimer, thrombin-antithrombin complex, prothrombin fragments 1+2, and hematocrit levels (p=0.318, 0.722, 0.743, 0.324, and 0.065, respectively). Conclusion: Significant increase in the parameters that reflect activation of coagulation, such as sEPCR, thrombin-antithrombin complex, prothrombin fragments 1+2, and D-dimer, reflects the presence of a basal condition that leads to a tendency toward thrombosis development in ET and PV when compared to healthy controls.
  • Thumbnail Image
    Item
    Intrathecal Methotrexate-Induced Posterior Reversible Encephalopathy Syndrome (PRES)
    (2014) Guler, Tulay; Cakmak, Ozden Yener; Toprak, Selami Kocak; Kibaroglu, Seda; Can, Ufuk; 24764745