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    Multicenter retrospective analysis regarding the clinical manifestations and treatment results in patients with hairy cell leukemia: twenty-four year Turkish experience in cladribine therapy
    (Wiley, 2015) Hacioglu, Sibel; Bilen, Yusuf; Eser, Ali; Sivgin, Serdar; Gurkan, Emel; Yildirim, Rahsan; Aydogdu, Ismet
    In this multicenter retrospective analysis, we aimed to present clinical, laboratory and treatment results of 94 patients with Hairy cell leukemia diagnosed in 13 centers between 1990 and 2014. Sixty-six of the patients were males and 28 were females, with a median age of 55. Splenomegaly was present in 93.5% of cases at diagnosis. The laboratory findings that came into prominence were pancytopenia with grade 3 bone marrow fibrosis. Most of the patients with an indication for treatment were treated with cladribine as first-line treatment. Total and complete response of cladribine was 97.3% and 80.7%. The relapse rate after cladribine was 16.6%, and treatment related mortality was 2.5%. Most preferred therapy ( 95%) was again cladribine at second-line, and third line with CR rate of 68.4% and 66.6%, respectively. The 28-month median OS was 91.7% in all patients and 25-month median OS 96% for patients who were given cladribine as first-line therapy. In conclusion, the first multicenter retrospective Turkish study where patients with HCL were followed up for a long period has revealed demographic characteristics of patients with HCL, and confirmed that cladribine treatment might be safe and effective in a relatively large series of the Turkish study population. Copyright (C) 2014 John Wiley & Sons, Ltd.
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    Splenic Marginal Zone Lymphoma in Turkey: Association with Hepatitis B Instead of Hepatitis C Virus as an Etiologic and Possible Prognostic Factor - A Multicenter Cohort Study
    (2020) Okay, Müfide; Aslan, Tuncay; Özdemir, Evren; Üner, Ayşegül; Ayhan, Arzu; Güngör, Elif Sena; Uysal, Ayşe; Alayvaz, Nevin; Yıldızhan, Esra; Ağıt, Abdullah; Dal, Mehmet Sinan; Korkmaz, Serdal; Namdaroğlu, Sinem; Sivgin, Serdar; Akgün Çağlıyan, Gülsüm; Demircioğlu, Sinan; Barışta, İbrahim; Özhamam, Esra; Vural, Filiz; Eser, Bülent; Özet, Gülsüm; Yıldırım, Rahşan; Doğu, Mehmet; Berber, İlhami; Erkurt, Mehmet Ali; Malkan, Ümi?T Yavuz; Altuntaş, Fevzi; Büyükaşık, Yahya
    Abstract: Objective: Chronic antigenic stimulation is frequently blamed in the pathogenesis of extranodal marginal zone lymphomas including splenic marginal zone lymphoma (SMZL). Chronic hepatitis C is frequently observed in SMZL patients in some geographical regions. However, these reports are largely from North America and Europe, and data from other countries are insufficient. In this multicenter study we aimed to identify the clinical characteristics of SMZL patients in Turkey, including viral hepatitis status and treatment details. Materials and Methods: Data were gathered from participating centers from different regions of Turkey using IBM SPSS Statistics 23 for Windows. Hepatitis B virus surface antigen (HBsAg), anti-HBs antibody, anti-HB core antigen antibody (anti-HBcAg), HB viral load, anti-hepatitis C virus (HCV) antibody, HCV viral load results were analyzed. Results: One hundred and four patients were reported. Hepatitis C virus positivity was observed in only one patient. However, hepatitis B virus surface antigen (HBsAg) positivity was observed in 11.2% and HBsAg and/or anti-HB core antigen antibody (anti-HBcAg) positivities were seen in 34.2% of the patients. The median age was 60 years (range=35-87). Median follow-up duration was 21.2 months (range=00.2-212; 23.2 months for surviving patients). Median overall survival was not reached. Estimated 3-year and 10-year survival rates were 84.8% and 68.9%, respectively. Older age, no splenectomy during follow-up, platelet count of <90x103/µL, lower albumin, higher lactate dehydrogenase, higher ?2-microglobulin, and HBsAg positivity were associated with increased risk of death. Only albumin remained significant in multivariable analysis. Conclusion: These results indicate that hepatitis B virus may be a possible risk factor for SMZL in our population. It may also be an indirect prognostic factor.
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    Therapeutic plasma exchange in patients with thrombotic thrombocytopenic purpura: A retrospective multicenter study
    (Pergamon-Elsevier Science Ltd, 2013) Korkmaz, Serdal; Keklik, Muzaffer; Sivgin, Serdar; Yildirim, Rahsan; Tombak, Anil; Kaya, Mehmet Emin; Acik, Didar Yanardag
    Thrombotic thrombocytopenic purpura (TIT) is a particular form of thrombotic microangiopathy typically characterized by thrombocytopenia, microangiopathic hemolytic anemia, fever, neurological abnormalities, and renal dysfunction. UP requires a rapid diagnosis and an adapted management in emergency. Daily sessions of therapeutic plasma exchange (TPE) remain the basis of management of UP. Also, UP is a rare disease that is fatal if it is not treated. TPE has resulted in excellent remission and survival rates in UP patients. Aim: We aimed to present our experience in 163 patients with UP treated with TPE during the past 5 years from 10 centers of Turkey. Patients and methods: One hundered and sixty-three patients with UP treated with TPE during the past 5 years from 10 centers of Turkey were retrospectively evaluated. TPE was carried out 1-1.5 times plasma volume. Fresh frozen plasma (FFP) was used as the replacement fluid. TPE was performed daily until normalization of serum lactate dehydrogenase (LDH) and recovery of the platelet count to >150 x 10(9)/dL. TPE was then slowly tapered. Clinical data, the number of TPE, other given therapy modalities, treatment outcomes, and TPE complications were recorded. Results: Fifty-eight percent (95/163) of the patients were females. The median age of the patients was 42 years (range; 16-82). The median age of male patients was significantly higher than female (53 vs. 34 years; p < 0.001). All patients had thrombocytopenia and microangiopathic hemolytic anemia. At the same time, 82.8% (135/163) of patients had neurological abnormalities, 78.5% (128/163) of patients had renal dysfunction, and 89% (145/163) of patients had fever. Also, 10.4% (17/163) of patients had three of the five criteria, 10.4% (17/163) of patients had four of the five criteria, and 6.1% (10/163) of patients had all of the five criteria. Primary UP comprised of 85.9% (140/163) of the patients and secondary UP comprised of 14.1% (23/163) of the patients. Malignancy was the most common cause in secondary UP. The median number of TPE was 13 (range; 1-80). The number of TPE was significantly higher in complete response (CR) patients (median 15.0 vs. 3.5; p < 0.001). CR was achieved in 85.3% (139/163) of the patients. Similar results were achieved with TPE in both primary and secondary UP (85% vs. 87%, respectively; p = 0.806). There was no advantage of TPE + prednisolone compared to TPE alone in terms of CR rates (82.1% vs. 76.7%; p = 0.746). CR was not achieved in 14.7% (24/163) of the patients and these patients died of TTP related causes. There were no statistical differences in terms of mortality rate between patients with secondary and primary TIT [15% (21/140) vs. 13% (3/23); p = 0.8061. But, we obtained significant statistical differences in terms of mortality rate between patients on TPE alone and TPE + prednisolone [14% (12/86) vs. 3% (2/67), p < 0.001]. Conclusions: TPE is an effective treatment for UP and is associated with high CR rate in both primary and secondary UP. Thrombocytopenia together with microangiopathic hemolytic anemia is mandatory for the diagnosis of UP and if these two criteria met in a patient, TPE should be performed immediately. (C) 2013 Elsevier Ltd. All rights reserved.