Browsing by Author "Ozkocaman, Vildan"

Filter results by typing the first few letters
Now showing 1 - 2 of 2
  • No Thumbnail Available
    Item
    Castleman Disease: A Multicenter Case Series from Turkey
    (2022) Gunduz, Eren; Kirkizlar, Hakki Onur; Umit, Elif Gulsum; Gulsaran, Sedanur Karaman; Ozkocaman, Vildan; Ozkalemkas, Fahir; Candar, Omer; Elverdi, Tugrul; Kucukyurt, Selin; Paydas, Semra; Ceneli, Ozcan; Karakus, Sema; Maral, Senem; Ekinci, Omer; Ipek, Yildiz; Kis, Cem; Guven, Zeynep Tugba; Akdeniz, Aydan; Celkan, Tiraje; Kucukdiler, Ayse Hilal Eroglu; Cagliyan, Gulsum Akgun; Sengoz, Ceyda Ozcelik; Karatas, Ayse; Bulduk, Tuba; Ozcan, Alper; Apak, Fatma Burcu Belen; Canbolat, Aylin; Kartal, Ibrahim; Oren, Hale; Toret, Ersin; Ozdemir, Gul Nihal; Ozturk, Sule Mine Bakanay; 35176839
    Objective: Castleman disease (CD) is a rare disease also known as angiofollicular lymph node hyperplasia. The two main histological subtypes are the hyaline vascular and plasma cell variants. It is further classified as unicentric CD (UCD) or multicentric CD (MCD) according to the anatomical distribution of the disease and the number of lymph nodes involved. The aim of this multicenter study was to evaluate all cases of CD identified to date in Turkey to set up a national registry to improve the early recognition, treatment, and follow-up of CD. Materials and Methods: Both adult (n=130) and pediatric (n=10) patients with lymph node or involved field biopsy results reported as CD were included in the study. Patients' demographic information, clinical and laboratory characteristics, imaging study results, treatment strategies, and clinical outcomes were evaluated retrospectively. Results: A total of 140 patients (69 male and 71 female) with a diagnosis of UCD (n=73) or MCD (n=67) were included. The mean age was 39 years in the UCD group and 47 years in the MCD group. Female patients were more common in the UCD group. The most common histological subtype was hyaline vascular for both UCD and MCD patients. Asymptomatic patients were more common in the UCD group. Anemia, elevations of acute phase reactants, and hypoalbuminemia were more common in the MCD group. The most commonly used treatment strategies for UCD were surgical excision, rituximab, and radiotherapy, respectively. All UCD patients were alive at a median of 19.5 months of follow-up. The most commonly used treatment strategies for MCD were methyl prednisolone, R-CHOP, R-CVP, and rituximab. Thirteen MCD patients had died at a median of 34 months of follow-up. Conclusion: This study is important in presenting the patient characteristics and treatment strategies for CD from Turkey, with the potential of increasing awareness about CD. Treatment data may help in making decisions, particularly in countries that do not have access to siltuximab. However, larger prospective studies are needed to make definitive conclusions.
  • No Thumbnail Available
    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.