Browsing by Author "Unal, Ali"
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Publication Evolution of clinical characteristics of patients with paroxysmal nocturnal hemoglobinuria treated with eculizumab in Turkey: A multicenter retrospective analysis.(E-century Publishing Corp, 2021-01-01) Karadag, Fatma Keklik; Yenerel, Mustafa Nuri; Yilmaz, Mehmet; Uskudar, Hava; 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; Ilhan, Osman; Saydam, Guray; Sahin, Fahri; Ozkocaman, Vildan; ÖZKOCAMAN, VİLDAN; Ali, Ridvan; ALİ, RIDVAN; Bursa Uludağ Üniversitesi/Tıp Fakültesi; 0000-0003-2687-9167; 0000-0002-7798-4349; 0000-0001-8605-8497; 0000-0003-1977-0104; 0000-0002-8902-1283; 0000-0002-2176-4371; 0000-0003-0604-6475; 0000-0003-0757-9206; 0000-0001-7717-5827; 0000-0002-7195-1845; 0000-0001-9178-2850; 0000-0003-2824-1044; 0000-0001-7842-9702; AAA-2012-2021; S-4300-2019; GLU-6163-2022; HRC-6282-2023; ABH-5764-2020; W-2951-2017; ABE-4485-2021; ITT-2117-2023; IZQ-0529-2023; W-3827-2017; GLQ-6094-2022; HKM-4739-2023; ABC-8182-2021Paroxysmal 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.Publication Study for the diagnostic screening of paroxysmal nocturnal hemoglobinuria in older patients with unexplained anemia and/or cytopenia(Clin Lab Publ, 2020-01-01) Ozdemir, Zehra N.; Ilhan, Osman; Özet, Gülsüm; Falay, Mesude; Yenerel, Mustafa; Tuğlular, Tulin; Turgut, Mehmet; Güvenç, Birol; Unal, Ali; Ayyıldız, Orhan; Andıç, Neslihan; Hacihanefioğlu, Abdullah; Şahin, Fahri; Şencan, Mehmet; Özsan, Güner H.; Yıldırım, Rahşan; Tiftik, Eyüp N.; Tombak, Anil; Salim, Ozan; Kaya, Emin; Akay, Olga M.; Okan, Vahap; Pehlivan, Mustafa; Saydam, Güray; Ali, Ridvan; ALİ, RIDVAN; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Hematoloji Anabilim Dalı.; GXD-8209-2022Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired hematopoietic stem cell disease that may lead to weakness and death of patients, if unrecognized and untreated. Although consensus guidelines were reviewed recently for the diagnostic screening of PNH with multi-parameter flow cytometry (FCM), until now, no study has investigated the efficiency of such clinical indications in older patients.Methods: Overall, 20 centers participated in the study and a total of 1,689 patients were included, 313 of whom were at geriatric age and 1,376 were aged 18 - 64 years. We evaluated the efficiency of consensus clinical indications for PNH testing using FCM in peripheral blood samples and compared the results of older patients and patients aged 18 - 64 years.Results: PNH clones were detected positive in 7/313 (2.2%) of the older patients. Five (74.4%) of the patients with PNH clones had aplastic anemia, 1 had unexplained cytopenia, and 1 patient had myelodysplastic syndrome (MDS) with refractory anemia. PNH clones were not detected in any older patients who were screened for unexplained thrombosis, Coombs (-) hemolytic anemia, hemoglobinuria, and others (e.g., elevated lactate dehydrogenase (LDH), splenomegaly). We detected PNH clones in 55/1376 (4%) samples of the patients aged under 65 years. Forty-two (76.4%) patients with PNH clones had aplastic anemia, 2 patients had Coombs (-) hemolytic anemia, 3 patients had unexplained cytopenia, 1 patient had MDS with refractory anemia, 1 patient had hemoglobinuria, and 6 (10.9%) had others (e.g., elevated LDH, splenomegaly). PNH clones were not detected in any patients who were screened for unexplained thrombosis. There was no statistical difference between the geriatric population and patients aged 18 - 64 years in terms of clinical indications for PNH screening with FCM (p = 0.49).Conclusions: Our results showed that the current clinical indications for PNH screening with FCM were also efficient in older patients. We suggest that older patients with unexplained anemia, myelodysplastic syndrome with refractory anemia, and unexplained cytopenia should be screened for PNH with FCM to identify patients who would benefit from treatment.