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    A Rare Nephrotic Syndrome Related to Chronic Lymphocytic Leukemia: Focal Segmental Glomerulosclerosis
    (Springernature, 2022) Karakus, Volkan; Atas, Unal; Uzuntas, Sahnura; Dere, Yelda; Meteoglu, Ibrahim
    Chronic lymphocytic leukemia (CLL) is a hematological disease characterized by the proliferation of monoclonal B-lymphocytes. Although autoimmune complications such as autoimmune hemolytic anemia and immune thrombocytopenia are common in CLL patients, nonhematological autoimmune complications are rather rare. The most common renal involvements are membranoproliferative glomerulonephritis and minimal change disease. Focal segmental glomerulosclerosis (FSGS) is predominantly associated with Hodgkin's lymphoma among hematological malignancies. FSGS associated with CLL is rarely reported in the literature, with a poor understanding of the common pathophysiology and a very limited experience with this co-occurrence. Although Rai Stage 1/Binet Stage B CLL, our 61-year-old case, who was diagnosed with secondary FSGS, which is a very rare complication, was treated with fludarabine, cyclophosphamide, and rituximab (FCR) combination. Following the treatment, a complete response was achieved about CLL, and the patient, whose renal findings recovered, is in remission and under follow-up for six years. Although the mechanisms between CLL and autoimmune complications are not fully elucidated, it is usually related to immune disorders like an abnormal T-cell response and polyclonal antibody production. While FSGS is very rare in lymphoma, its co-existence with CLL is reported only in a limited number of case reports. Steroids may be used in these patients; however, in cases not responding to steroids, treatment of the underlying CLL is required.
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    Different clinical courses with the same findings: two cases of paroxysmal nocturnal hemoglobinuria presenting with thrombocytopenia
    (2021) Karakuş, Volkan; Kaya, Egemen; Dere, Yelda; Şahin, Fahri
    Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal stem cell disease that manifests with chronic intravascular hemolysis, thrombosis, and bone marrow failure. Various degrees of cytopenias accompany the disease. Although laboratory and clinical findings are similar, the disease may show different courses and require different treatments. Herein, we report two different courses of PNH with similar clinical and laboratory findings.
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    Serum soluble Fas-ligand levels and flow-mediated vasodilation in patients undergoing peritoneal dialysis
    (2022) Huddam, Bülent; Azak, Alper; Karakuş, Volkan; Alp, Alper; Genek, Dilek Gibyeli; Koçak, Meral Gülay Kadıoğlu; Dere, Yelda; Soysal, Dilek Ersil; Duranay, Murat
    Flow-mediated vasodilation (FMD) has been demonstrated to be a useful, non-invasive tool for the detection ofendothelialdysfunction in atherosclerotic cardiovascular disease, the leading cause of mortality in end-stage kidney disease. The Fas/Fas ligand system of apoptosis resulting from activation of the caspase cascade- contributes to the pathophysiology of atherosclerosis. This ‘apoptotic’ system plays a central role in immune homeostasis. Vascular endothelial cells and inflammatory cells are the main resources of the Fas ligand. In this study, we aimed to investigate the role of soluble Fas ligand (sFasL) as a marker of FMD in peritoneal dialysis (PD) patients. Methods. A total of 43 patients undergoing maintenance PD and 40 healthy donors were enrolled in this cross-sectional observational study. Demographics, anthropometric measurements and clinical examinations were obtained. Endothelial function was evaluated by FMD of the brachial artery with high-resolution ultrasonography. Serum sFasL concentrations were measured with an enzyme-linked immunosorbent assay kit. Results. The enrolled partisipants were devited on 2 groups: PD patients who had been treated at least 12 weeks (group 1; mean age 41±14 years, M/F: 22/21) and gender matched 40 healthy controls (group 2; mean age 50±12 years, M/F: 19/20). The forearm FMD and serum sFasL levels were significantly lower in PD patients (3.95±2.01 vs 8.83 ± 6.17; p<0.001 and 54 ± 24 vs 73 ± 30; p=0.001). Forearm FMD was correlated with sFasL (r=0.289; p=0.008), age, BMI and uric acid (r= 0,32; p=0.003, respectively), hemoglobin (r= 0,293; p=0.007), calcium (r= 0,26; p=0.016), phosphate (r=- 0,250; p=0.023), magnesium (r= 0,255; p=0.020), 24 h SBP (r=- 0,257; p=0.019), creatinine and iPTH (r=- 0.50 and r=- 0,45; p[removed]
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    Survival outcomes of hypomethylating agents maintenance therapy in new diagnosed AML patients: Real experience data
    (2022) Karakuş, Volkan; Maral, Senem; Kaya, Egemen; Gemici, Aliihsan; Dere, Yelda; Sevindik, Ömür Gökmen
    OBJECTIVE: Acute myeloid leukemia (AML) is a hematological malignancy that frequently affects elderly population. With introducing the hypomethylating agents (HMAs) in elderly AML treatment, survival rates and quality of life have improved. However, long-term management in elderly and frail patients is still a challenge. In the present study, we aimed to determine whether HMA maintenance therapy is required until disease progression in frail and elderly AML patients by examining with a real-life data. METHODS: In a multicenter study, we analyzed non-promyelocytic elderly AML patients who were treated with first-line azacitidine or decitabine monotherapy in two different groups, retrospectively. While patients were treated with HMA until progression in the maintenance group, 6+3 cycles of azacitidine or decitabine were administered as a standard care of elderly AML patients in the non-maintenance group. Survival outcomes were compared between the groups. RESULTS: HMA therapy was maintained until progression in 20 patients, and HMA therapy was terminated after 6+3 cycles in 21 patients. Patients received a median of 6 (1–14) HMA cycles during follow-up time. The median 7.5 months of overall survival were observed (2–17 months) in maintenance and 3 months (1–13 months) in non-maintenance groups (p=0.001). CONCLUSION: Despite long-term exposure to HMA may appear as a risk factor for complications and toxicities in elderly and frail AML patients, the maintenance of therapy until disease progression provides a significant survival advantage. Therefore, we suggest that HMA therapy should continue until disease progression regardless the sort of HMA.