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    Eltrombopag for the Treatment of Immune Thrombocytopenia: The Aegean Region of Turkey Experience
    (Galenos Yayincilik, 2015) Ozdemirkiran, Fusun; Payzin, Bahriye; Kiper, H. Demet; Kabukcu, Sibel; Cagliyan, Gulsum Akgun; Kahraman, Selda; Sevindik, Omur Gokmen; Ceylan, Cengiz; Kadikoylu, Gurhan; Sahin, Fahri; Keskin, Ali; Arslan, Oyku; Ozcan, Mehmet Ali; Gorgun, Gulnur; Bolaman, Zahit; Buyukkececi, Filiz; Bilgir, Oktay; Alacactoglu, Inci; Vura, Filiz; Tombuloglu, Murat; Gokgoz, Zafer; Saydam, Güray
    Objective: Immune thrombocytopenia (ITP) is an immune-mediated disease characterized by transient or persistent decrease of the platelet count to less than 100x109/L. Although it is included in a benign disease group, bleeding complications may be mortal. With a better understanding of the pathophysiology of the disease, thrombopoietin receptor agonists, which came into use in recent years, seem to be an effective option in the treatment of resistant cases. This study aimed to retrospectively assess the efficacy, long-term safety, and tolerability of eltrombopag in Turkish patients with chronic ITP in the Aegean region of Turkey. Materials and Methods: Retrospective data of 40 patients with refractory ITP who were treated with eltrombopag in the Aegean region were examined and evaluated. Results: The total rate of response was 87%, and the median duration of response defined as the number of the platelets being over 50x10(9)/L was 19.5 (interquartile range: 5-60) days. In one patient, venous sinus thrombosis was observed with no other additional risk factors due to or related to thrombosis. Another patient with complete response and irregular follow-up for 12 months was lost due to sudden death as the result of probable acute myocardial infarction. Conclusion: Although the responses to eltrombopag were satisfactory, patients need to be monitored closely for overshooting platelet counts as well as thromboembolic events.
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    First Case of FLT3-Tyrosine Kinase Domain Mutant Acute Myeloid Leukemia With Unusual Onset as Isolated Bilateral Testicular Myeloid Sarcoma
    (Springernature, 2024) Erol, Veysel; Cagliyan, Gulsum Akgun; Ufuk, Furkan; Demir, Derya
    Testicular myeloid sarcoma (TMS) is a challenging pathology often posing diagnostic difficulties due to the poorly differentiated nature of tumor cells at the initial presentation. The delay in diagnosis significantly impacts patient life expectancy, emphasizing the need for prompt identification and treatment initiation. In certain cases, the presence of the Fms-like tyrosine kinase ( FLT3 ) mutation adds complexity to the disease, requiring tailored therapeutic approaches. In this report, we present a unique case of bilateral TMS with FLT3 tyrosine kinase domain ( TKD ) mutation. The patient exhibited an aggressive clinical course, initially misdiagnosed with orchitis during the initial evaluation. Subsequent reevaluation of the testicular biopsy at a second center led to an accurate diagnosis, highlighting the importance of thorough examination in challenging cases. Given the emerging significance of FLT3 mutations in myeloid sarcomas, comprehensive testing for all FLT3 variants is crucial to determine the appropriate treatment modality. This case underscores the need for increased awareness among healthcare professionals regarding the diagnostic nuances and potential genetic variations associated with TMS. Furthermore, the inclusion of tyrosine kinase inhibitors, such as midostaurin or gilteritinib, especially in the presence of FLT3 mutations, may significantly impact treatment outcomes. This report contributes to the growing body of literature on TMS and highlights the importance of considering FLT3 mutations in the diagnostic and therapeutic decision -making process for improved patient care.
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    Nodular lymphocyte predominant Hodgkin's lymphoma in daily practice: A multicenter experience
    (Wiley, 2018) Gemici, Aliihsan; Aydogdu, Ismet; Terzi, Hatice; Sencan, Mehmet; Aslan, Alma; Kaya, Ali Hakan; Dal, Mehmet Sinan; Akay, Meltem Olga; Dogu, Mehmet Hilmi; Ayyildiz, Orhan; Sahin, Fahri; Cagliyan, Gulsum Akgun; Yilmaz, Mehmet; Gokgoz, Zafer; Bilen, Yusuf; Demir, Cengiz; Sevindik, Omur Gokmen; Korkmaz, Serdal; Eser, Bulent; Altuntas, Fevzi
    Nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL) is a rare subtype of Hodgkin's lymphoma. In this study, we aimed to investigate the clinical features and therapeutic outcomes of patients with NLPHL who were diagnosed at different institutes in Turkey. We retrospectively reviewed the records of the patients diagnosed with NLPHL. Adult patients who were diagnosed after 2005 with histological confirmation were selected for the study. Forty-three patients were included in the study. Median age of patients was 37.5years (18-70) at the time of diagnosis. About 60.5% patients were diagnosed as stage I and II NLPHL, and remaining 39.5% had stage III and IV disease. Median follow-up was 46months. During follow-up, none of the patients died. Seven patients relapsed or progressed after initial therapy at a median of 12months. Five of 7 relapsed/refractory patients (71.4%) were salvaged with chemotherapy only (DHAP, ICE), and the remaining 2 (28.6%) were salvaged with chemoimmunotherapy. All of relapsed/refractory patients were able to achieve complete remission after salvage therapy. Lactate dehydrogenase levels were significantly higher in patients with progressive disease compared with nonprogressive disease. Our study showed an excellent outcome with all patients alive at last contact with a median follow up of 46months despite a wide range of different therapeutic approaches. All relapsed and refractory patients were successfully salvaged despite a low frequency of patients received immunotherapy in conjunction with chemotherapy. Our results suggest that immunotherapy may be reserved for further relapses.
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    Relationship Between Endocrinopathies and Ferritin Levels in Adult Turkish Patients with Beta Thalassemia Major: A Single-Center Experience
    (Erciyes Univ Sch Medicine, 2021) Cagliyan, Gulsum Akgun; Yaylali, GOzin Fidan; Soyer, Nur; Hacioglu, Sibel; Cagliyan, Omer; Guler, Nil
    Objective: Despite regular transfusions and iron-chelation therapies, endocrine complications still remain an important cause of morbidity in patients with beta thalassemia major (BTM). We aimed to evaluate the relationship between endocrine complications and serum ferritin levels in adult patients with BTM. Materials and Methods: The endocrine test results of adult patients with BTM were retrospectively reviewed. Normality testing was performed using the Shapiro-Wilk test. For the analysis, we used an independent samples t-test when parametric test assumptions were met: otherwise we used Mann-Whitney U test. A p value of <0.05 was considered statistically significant. Results: A total of 66 adult patients with BTM. (female: 56.1%, male: 43.9%, mean age: 25.8 +/- 6.6 years) were evaluated. The mean ferritin level was 1504.9 +/- 861 ng/ml (range 304-5464 ng/ml). of the patients, 60.6% had endocrinopathy. The rates of hypogonadism was 51.5%, hypothyroidism was 16.7% (subclinical hypothyroidism: 13.6%, central hypothyroidism: 3.03%), hypoparathyroidism was 10.6%, diabetes mellitus was 7.6%, vitamin D insufficiency was 40.9%, vitamin D deficiency was 33.3%, low bone mass was 56.1%, and that of low IGF-1 was 57.6%. We found a significant relationship between ferritin levels and the presence of hypogonadism, low bone mass, and low IGF-1 levels (p<0.001). Patients used higher doses of deferasirox due to iron overload (p<0.001). Patients needed higher deferasirox doses to decrease ferritin levels. Conclusion: Among adult patients with BTM, endocrinopathies were prevalent in patients with elevated ferritin levels. Patients with BTM should undergo regular testing for endocrine disorders, and ferritin levels must be kept under control.
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    The Turkish experience with therapeutic plasma exchange: A national survey
    (Pergamon-Elsevier Science Ltd, 2019) Korkmaz, Serdal; Medeni, Serife Solmaz; Demirkan, Fatih; Besisik, Sevgi Kalayoglu; Dadin, Senem Altay; Cagliyan, Gulsum Akgun; Hacioglu, Sibel Kabukcu; Sari, Ismail; Sahin, Deniz Goren; Arat, Mutlu; Dagdas, Simten; Ozet, Gulsum; Kutlu, Nermin; Akyol, Tulay Karaagac; Ozcebe, Osman Ilhami; Teke, Hava Uskudar; Unal, Demet Kiper; Guner, Naile; Tombak, Anil; Celik, Halil; Bay, Ilker; Kiki, Ilhami; Ozgur, Gokhan; Erkurt, Mehmet Ali; Ozatli, Duzgun; Meletli, Ozgur; Demircioglu, Sinan; Demir, Cengiz; Kurtoglu, Erdal; Vural, Filiz; Tobu, Mahmut; Karakus, Abdullah; Ayyildiz, Orhan; Dal, Mehmet Sinan; Ozturk, Berna Afacan; Albayrak, Murat; Ocakci, Serkan; Bolaman, Zahit; Sonmez, Mehmet; Karakus, Volkan; Sevindik, Omur Gokmen; Berber, Ilhami; Dogu, Mehmet Hilmi; Gulturk, Emine; Ulas, Turgay; Payzin, Bahriye; Kuku, Irfan; Cagirgan, Seckin; Altuntas, Fevzi
    Therapeutic plasma exchange (TPE) is used to treat more than 60 diseases worldwide and has drawn growing interest. Little is known about the current situation of TPE activity in Turkey, so we developed a survey to obtain information about this timely topic. We collected data on TPE from 28 apheresis units throughout Turkey. We performed a total of 24,912 TPE procedures with 3203 patients over the past decade. Twenty years ago, the majority of procedures were performed for neurological and hematological disorders, and today, most TPE procedures are done for the same reasons. The only historical change has been an increase in TPE procedures in renal conditions. Currently, renal conditions were more frequently an indication for TPE than rheumatic conditions. Fresh frozen plasma was the most frequently used replacement fluid, followed by 5% albumin, used in 57.9% and 34.6% of procedures, respectively. The most frequently used anticoagulants in TPE were ACD-A and heparin/ACD-A, used with 1671 (52.2%) and 1164 (36.4%) patients, respectively. The frequency of adverse events (AEs) was 12.6%. The most common AEs were hypocalcemia-related symptoms, hypotension, and urticaria. We encountered no severe AEs that led to severe morbidity and mortality. Overall, more than two thirds of the patients showed improvement in the underlying disease. Here, we report on a nationwide survey on TPE activity in Turkey. We conclude that there has been a great increase in apheresis science, and the number of TPE procedures conducted in Turkey has increased steadily over time. Finally, we would like to point out that our past experiences and published international guidelines were the most important tools in gaining expertise regarding TPE.