Browsing by Author "Akyol, Sefika"

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Clinical Spectrum of Primary Hemophagocytic Lymphohistiocytosis: Experience of Reference Centers in Central and Southeast Anatolia
(Springer, 2024) Akyol, Sefika; Yilmaz, Ebru; Tokgoz, Huseyin; Karaman, Kamuran; Pekpak, Esra; Ozcan, Alper; Unal, Ekrem
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease, with a high mortality if left untreated. In addition, the disease has unique diagnostic challenges. Therefore, despite the existing guidelines on management, current clinical practice data is informative on the course and outcome. Herein, a retrospective chart review study was conducted through the collaboration of six centers, located in central and southeastern Turkiye. The demographical data, laboratory results, and treatment outcomes were evaluated. Eighty-three patients were enrolled in the study. The mean age was 2 years, whereas the median age was 8 months with a range of a minimum of 1 week and a maximum of 12.6 years. Consanguineous marriage, history of sibling death, and familial history of similar disease were determined in 72.2% (n:60), 34.9% (n:29), and 39.8% (n:33) of the patients, respectively. The most common presentation was fever, followed by hepatosplenomegaly on admission. Disease-causing familial HLH variants were identified in 60.2% (n:50) of the patients. Hematopoietic stem cell transplantation (HSCT) was performed in 39.7% (n:33) of the cohort. The 2-year overall survival (OS) rate was 62.4% for the whole group. Comparing the patients who received HSCT and those who did not; the HSCT group had a 2-year OS of 84.7%, which was significantly better than patients who did not receive HSCT had a 2-year OS of 47.1% (p:0.001). Despite the improvement in HLH diagnostics and treatment options over the last decade, early death remains a leading problem for the survival of these patients. Therefore, appropriate assessment of the patients in experienced centers and HSCT are pivotal for better outcomes.
Rituximab in Pediatric B-Cell Non-Hodgkin Lymphoma: Clinical Outcomes and Prognostic Implications
(Galenos Publ House, 2025) Akyol, Sefika; Guzel, Turan; Ozcan, Alper; Karaman, Serap; Orhan, Mehmet Fatih; Uzel, Veysiye Hulya; Unal, Ekrem
Objective: B-cell Non-Hodgkin Lymphoma (B-NHL) is an aggressive malignancy in children requiring prompt multidisciplinary management. This retrospective cohort study aims to evaluate the clinical characteristics, treatment outcomes, and impact of rituximab (RTX) in pediatric B-NHL patients. Methods: We retrospectively analyzed 62 pediatric B-NHL patients treated at tertiary centers. Patient demographics, clinical presentation, histopathological subtypes, disease stage, treatment regimens, and survival outcomes were assessed. Event-free survival (EFS) and overall survival (OS) rates were analyzed based on lactate dehydrogenase (LDH) levels and RTX administration. Results: The mean age at diagnosis was 8.73 +/- 4.3 years, with a male predominance (79%). The most common histological subtype was Burkitt lymphoma (BL) (53.2%), followed by diffuse large B-cell lymphoma (DLBCL) (33.8%). Advanced-stage disease (III-IV) was observed in 74.1% of cases. RTX was administered in 72.5% of patients, with a mean of 5.1 +/- 2.7 doses. Febrile neutropenia (FEN) was noted in 74.1%, with intensive care unit (ICU) admission required for seven patients. Mortality was observed in 12 (19.3%) patients, including all patients with primary immunodeficiency (PID). The 5-year EFS for the entire cohort was 67.2%, and OS was 81.3%. Patients with LDH <400 U/L had superior 5-year EFS (88.9%) and OS (96.3%) compared to those with LDH >400 U/L (EFS: 49.6%, OS: 70.7%; p=0.004 and p=0.015, respectively). In RTX-treated patients without PID, EFS was 76.5% versus 73.2% in those without RTX, but the difference was not statistically significant (p=0.53). Conclusions: Although not statistically significant, EFS was found to be higher in the RTX-treated group, suggesting that adding RTX to standard chemotherapy regimens may improve survival, particularly for high-risk patients, though its benefit in low-risk cases remains uncertain. Despite improved survival, patients with PID had poor outcomes, likely due to increased infections and disseminated disease. Risk-adapted, targeted treatment strategies are essential for optimizing outcomes in pediatric B-NHL. Further large-scale, randomized controlled trials are needed to confirm the efficacy of RTX in different risk groups and to optimize treatment regimens for pediatric B-NHL.