Excess iron accumulation occurs mainly in organs such as reticuloendothelial cells, the pituitary gland, and the pancreas in beta-thalassemia because of blood transfusions. In the present study, it was aimed to investigate the relationship between T2* values on magnetic resonance imaging (MRI) and clinically diagnosed pituitary endocrinological disorders in children with thalassemia major. This study enrolled patients diagnosed with beta-thalassemia at pediatric hematology outpatient clinics. In the study, in addition to the medical history of the patients, routinely performed tests, including hemoglobin electrophoresis, routine biochemical tests, and tests for pubertal development (follicle-stimulating hormone (FSH), luteinizing hormone (LH), estradiol, testosterone, etc.), as well as iron deposition measured by hepatic MRI T2* (STAR) sequence, were retrospectively assessed. A total of 29 patients were enrolled. Hypothyroidism was detected in 34.6% (9/26) of patients, short stature in 37% (10/27), and pubertal retardation in 50% (14/28) of the patients. There was no significant correlation between hypothyroidism and pituitary MRI T2* values. No significant correlation was found between laboratory parameters and pituitary MRI examination. Although the sensitivity of T2* levels could rise above 80%, their specificity remained low. This is one of the major limitations of the pituitary MR T2* study for the prediction of short stature. The best lower cut-off level of MR T2* to predict short stature was found 14.6 ms. The diagnostic specificity pituitary MR examination levels for short stature were detected as low. Thus, the clinical standardization and validation of pituitary MR T2* values examination are needed before clinical follow-up and multifaceted studies are needed.

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 (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.

Background. Microbiologic confirmation of respiratory tract infections gained importance during the coronavirus disease 2019 (COVID-19) pandemic. This study retrospectively evaluated seasonal distribution, clinical presentation, and complications of respiratory viral infections (RVIs) other than COVID-19 in children with cancer during and after the pandemic lockdown. Methods. Two hundred and sixty-five inpatient and outpatient RVI episodes in 219 pediatric cancer patients confirmed by multiplex reverse transcriptase polymerase chain reaction (RT-PCR) panels from 13 centers were enrolled. Results. Eighty-six (32.5%) of the total 265 episodes occurred in 16 months corresponding to the lockdowns in Türkiye, and the remaining 67.5% in 10 months thereafter. Human rhinovirus/enterovirus (hRE) (48.3%) was the most common agent detected during and after lockdown. Parainfluenza virus (PIV) (23.0%), influenza virus (9.8%), and respiratory syncytial virus (RSV) (9.1%) were the other common agents. The 28.7% of episodes were lower respiratory tract infections (LRTIs), and complications and mortality were higher than upper respiratory tract infections (URTIs) (25.0% vs 5.3%). Bacteremia was identified in 11.5% of culture-drawn episodes. Treatment delay in one-third and death within four weeks after RVI in 4.9% of episodes were observed. Conclusion. During the pandemic, fewer episodes of RVIs occurred during the lockdown period. Respiratory viruses may cause complications, delays in treatment, and even death in children with cancer. Therefore, increased awareness of RVIs and rapid detection of respiratory viruses will benefit the prevention and, in some cases, abrupt supportive and some antiviral treatment of RVI in children with cancer.

Aim: This study aimed to compare treatment outcomes (mortality and relapse) between two pediatric acute lymphoblastic leukemia (ALL) protocols (TR-ALL BFM 2000 vs ALL-IC-BFM 2009) and to describe the demographic, clinical, and laboratory characteristics of patients treated at a single center (2010–2015). Methods: We retrospectively reviewed the medical records of 121 children (<18 years) diagnosed with ALL and treated with either TR-ALL BFM 2000 or ALL-IC-BFM 2009. Baseline characteristics, risk group, immunophenotype, protocol, relapse status, and survival status were recorded. Categorical variables were compared using the chi-square test or Fisher’s exact test, as appropriate (p < 0.05). Results: The cohort was 53.7% male, with a mean age at diagnosis of 7.55 ± 3.89 years. B-cell ALL accounted for 78.5% and T-cell ALL for 21.5%. The overall mortality rate was 11.6% (14/121), and relapse occurred in 5.8% (7/121) of patients. Mortality differed by risk group (SRG 6.5%, IRG 7.3%, HRG 23.5%; p = 0.036). Relapse was higher in T-cell ALL than in B-cell ALL (23.1% vs 1.1%; p = 0.001). Compared with ALL-IC-BFM 2009, TR-ALL BFM 2000 was associated with higher mortality (19.2% vs 5.8%; p = 0.022) and relapse (11.5% vs 1.4%; p = 0.019). Conclusion: In this single-center retrospective cohort study, the high-risk group was associated with higher mortality, and the T-cell immunophenotype was associated with higher relapse. Patients treated with ALL-IC-BFM 2009 had lower crude mortality and relapse rates than those treated with TR-ALL BFM 2000; however, these protocol comparisons should be interpreted cautiously, given potential confounding and limited event numbers.

Objectives: Hemoglobin disorders are quite heterogeneous in the Turkish population. Up to now, more than forty different beta thalassemia mutations and 60 hemoglobin variants have been characterized in the country. The aim of this study was to investigate genetic heterogeneity of HBB gene mutations in patients and their parents at Southeastern Anatolia in Turkey. Methods: Genomic DNA was isolated from 145 thalassemic patients’ blood samples and their parents in this study. Ten different HBB gene mutations HBB:c.-80T>A, HBB:c.17_18delCT, HBB:c.25_26delAA, HBB:c.92+1G>A, HBB:c.92+5G>C, HBB:c.92+6T>C, HBB:c.93-21G>A, HBB:c.135delC, HBB:c.315+1G>A, HBB:c.316-106C>G were screened by amplification refractory mutation system. Four Hb variants and some rare beta thalassemia mutation were characterized by DNA sequencing. Results: In this study, 97 homozygous and 48 compound heterozygous thalassemic patients were diagnosed by molecular genetic analyses. As a results, 18 β-thalassemia mutations and four abnormal hemoglobins; HBB:c.20A>T, HBB:c.364G>C, HBB:c.34G>A and HBB:c.208G>A were detected at Dicle University Hospital. Conclusions: In the results, HBB:c.93-21G>A is the most common mutation in the region. Three mutations [(HBB:c.93-21G>A), (HBB:c.25_26delAA) and (HBB:c.135delC)] account for about 58 per cent of all the point mutations. Except HBB:c.20A>T and HBB:c.364G>C, two silent Hb variants (HBB:c.34G>A and HBB:c.208G>A) were detected in this study. Hb Hamilton [β11 (GTT>ATT) Val>Ile] was seen first time in Turkey.

Background. Based on the information obtained so far, COVID- 19 is relatively mild in children. We will present a 6-month-old male patient infected with COVID -19 in April 2020, while receiving HLH 2004 chemotherapy protocol with the diagnosis of familial (Genetic / Primary) Hemophagocytic Lymphohistiocytosis (HLH). Case. Herein we present a case accompanied by a defective perforin gene defect in the primary HLH pathogenesis, Covid-19 infection with the presence of fever and hyperferritinemia, which was evaluated in favor of reactivation and the patient was given both the HLH-2004 chemotherapy protocol treatment and COVID -19 therapy as recommended by the guidelines. Our patient improved clinically and in terms of laboratory test results at the end of the 15th day of hospitalization and was discharged. Conclusions. It should be remembered that COVID-19 can be seen with different clinical manifestations in the pediatric age group, and COVID-19 tests should be recommended, especially in children with immunosuppression and fever.