Browsing by Author "Erdem, Fuat"

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    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.
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    Relationship between mannose-binding lectin and febrile neutropenia in acute leukemia patients
    (2020) Akan, Selcuk; Bakanay, Sule Mine; Bilen, Yusuf; Balcan, Baran; Erdem, Fuat
    Aim: Mannose-binding lectin (MBL) is an important component of the natural immune system. Its low levels have been linked to increased frequency of opportunistic infections. This study aimed to determine the association of serum MBL levels and duration of febrile neutropenia (FN) after cytotoxic chemotherapy in patients with acute leukemia. Material and Methods: Seventy patients aged 15-75 years with acute leukemia (40 AML (Acute myeloid leukemia), 30 ALL (Acute lymphoblastic leukemia)), and 30 age-matched healthy subjects were included in this study. Blood MBL levels were measured using ELISA Kit before chemotherapy (MBL1) and at FN (MBL2). Results: The MBL1 levels of the patient group (Median 466 ng/ml; interquartile range (iqr): 4507) were higher than that of the control group (Median 485 ng/ml; iqr: 1872), but this difference was statistically insignificant (p=0.92). During FN, MBL levels of 49 (70%) patients increased and 21 (30%) patients remained at the same level. The MBL2 levels (Median 772 ng/ml; iqr: 5870) of the patients were significantly higher than the MBL1 levels (p< 0.001). Patients with very low (<100 ng/ml) MBL2 levels had significantly longer FN duration than patients with normal (>1000 ng/ml) MBL2 levels (p=0.016). Discussion: Our results suggest that duration of FN is longer when MBL level is low especially less than 100 ng/ml. These patients seem to have the highest risk for infection-related morbidity and mortality and deserve interest for trials of MBL replacement.