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Malignant germ cell tumors (GT) arise from abnormal migration of primordial germ cells and are histologically identical whether they occur inside or outside of central nervous system (CNS) (1). They are divided into two heterogenous groups: germinomas and non-germinomatous germ cell tumors according to histological findings. Although the optimal treatment strategy remains a matter of debate, they generally respond well to surgery, radiotherapy, chemotherapy, or a combination of all and are characterized by a good survival rate (2). The surgical strategy for GCTs varies depending on the location of the tumor. Surgery is only a standard care option for low grade extracranial GCTs because it is unlikely to achieve negative tumor margins when the tumor is located inside CNS (2). Patients with relapsed or progressive despite initial chemotherapy are candidates for salvage therapy (3). Patients relapsing after definite treatment of locoregional disease are to be treated by stage adopted first line standard therapy for metastatic disease. Patients with primary advanced/metastatic disease failing one line of cisplatin based combination chemotherapy can benefit from high dose chemotherapy and stem cell rescue (4). Although stem cell transplantation following high dose chemotherapy can be beneficial, either two or three consecutive cycles of high dose chemotherapy with repetition after blood count recovery may contribute to overall survival rates.

Objective: Objective: Steroid-resistant acute graft-versus-host disease (srAGVHD) is the most important cause of morbidity and mortality after allogeneic stem cell transplantation. There are several treatment methods available, including mesenchymal stem cell (MSC) application. The aim of this study was to evaluate the results of MSC therapy performed in children with srAGVHD. Materials and Methods: MSC therapy was used in our center between November 2014 and December 2017 for 22 patients who developed srAGVHD. The patients were retrospectively evaluated in terms of treatment response and survival. Results: After application of MSCs, complete response was obtained in 45.5% of the subjects, partial response was obtained in 13.6%, and no response was obtained in 40.9%. We found that 45.5% of the patients were alive and 54.5% had died and our treatment results were similar to those in the literature. Response to MSC treatment was found to be the only prognostic marker affecting mortality. Conclusion: MSC application is a treatment method that can be used safely together with other treatment methods in srAGVHD, a condition that has a high mortality rate. There are almost no acute side effects. There are also no serious long-term side effects in the literature. Prospective randomized studies are required to obtain high-quality data.

Hodgkin`s lymphoma (HL) is highly curable disease in its early stages, but in advanced stages, it presents a dilemma when it becomes refractory or relapses after several rounds of chemotherapy. Brentuximab vedotin (BV) is an antibody-drug conjugate that targets the tumor necrosis receptor family protein member CD30 positive malignancies via an anti-CD30 monoclonal antibody linked to monomethyl auristatin-E. In adult and pediatric studies, it has been shown to be an effective salvage therapy for primary refractory HL or relapse after autologous stem cell transplant (ASCT). Between July 2012 and August 2017, we administered BV (1.8 mg/m2 every three weeks; 12 cycles totally) with doxorubucin, vinblastin, dacarbazine (AVD), rituximab + ifosfamide + carboplatin + etoposide (RICE), or bendamustine combination treatment in pediatric HL patients, who were previosuly treated for refractory or relapsed advanced stage HL before (seven patients) or after (one patient) ASCT in our center. After eight BV courses, one patient was able to undergo match unrelated donor (MUD) SCT. Another seven pediatric HL patients, who were not able to go into remission with any other classical HL chemotherapy protocols, received 4-6 courses of BV-AVD and/or RICE/bendamustine. All were able to undergo ASCT after negative positron emission tomography (PET) imaging results. After ASCT, we switched to BV as consolidation therapy until a total of 12 cycles was completed. Patients went into remission after a median 34 (range: 12-42) months from the start of BV treatment. BV is an encouraging, well- tolerated, and effective targeted therapy especially when combined with AVD or when alternated with another targeted therapy combination, including RICE, when needed.

Neuroblastoma (NB) is the most frequently diagnosed neoplasm during infancy and its incidence declines within the first 3-5 years of life. It can be rarely diagnosed in adolescents and young adults. Adolescents have advanced stage of disease, higher frequency of uncommon metastatic sites such as lungs, and worse outcomes. Herein, we describe an unusual case of NB in a 17-year-old adolescent presented with lung metastasis at diagnosis. The patient was diagnosed with stage IV NB. Thorax high-resolution computed tomography (HRCT) scan revealed irregular septal thickening with ground glass opacity consistent with pulmonary parenchymal metastases. After the first cycle of chemotherapy he developed pulmonary hemorrhage and respiratory distress. He required ventilation support and mechanical ventilation was started. Metastatic nodules were determined on second thorax HRCT. We lost the patient due to septic shock and multiple organ failure 2 months after diagnosis. In conclusion, adolescents with NB have unfavorable prognosis. These patients may have lung metastases at diagnosis. Therefore, detailed chest imaging at initial diagnosis is crucial.

Aim: To investigate the effects of anti‐human Leucocyte Antigen (HLA) antibody positivity on early hematopoietic stem cell transplantation (HSCT) results in patients with thalassemia major (TM). Methods: One hundred and twenty‐four HLA‐matched HSCTs were performed in patients with TM between 2015 and 2022. Ninety‐one patients were screened for anti‐HLA antibodies by testing panel reactive antigens (PRA). Demographic and transplantation characteristics of patients were recorded. The presence of PRA was tested with the Antibody Testing Assay (Luminex LIFECODES HLA Antibody Identification System). Results: The number of PRA‐positive patients was 54. There was no relationship between acute graft versus host disease (GVHD), chronic GVHD, grade of GVHD, and viral reactivation of the patients. However, platelet engraftment took around 3 days longer in the PRA‐positive group (p = 0.05). The median number of erythrocyte transfusions was significantly higher in PRA‐positive patients in the post‐transplant period (p = 0.003), as was the median number of platelet transfusions (p = 0.003). Treosulfan conditioning increased the stable mixed chimerism (MC) rate by 3.8‐fold (p = 0.011). In contrast, reduced rates of MC were found in patients who received matched unrelated donor cells or peripherally derived stem cells (p = 0.011 and p = 0.039, respectively) in the posttransplantation period in TM patients. PRA‐positivity did not affect MC (p = 0.478). However, 80% of patients who had primary graft failure (n = 5; p = 0.59) and 75% of patients who died (n = 4) were PRA positive (p = 0.64), but these results were statistically insignificant due to the low number of patients. Conclusion: Anti‐HLA antibodies primarily delayed platelet engraftment in TM patients and increased the erythrocyte and thrombocyte transfusion requirements. Although PRA positivity was more common in patients with primary graft failure or who died, there was no statistically significant impact of PRA positivity on chimerism, acute or chronic GVHD, viral activation, or mortality rates. [ABSTRACT FROM AUTHOR]

Background: BKV‐HC is one of the most significant complications of HSCT. This retrospective study aimed to determine the frequency of BKV‐HC in pediatric patients undergoing HSCT, detect the associated risk factors for the development of BKV‐HC, and explore the effects of post‐transplantation Cy use. Methods: Three hundred twenty‐seven patients (girls: 121, boys: 206) were analyzed according to sex, conditioning regimen, transplantation type, donor relatedness, stem cell source, the presence and grade of aGVHD, CMV co‐existence, and Cy use. Results: Multivariate analysis confirmed the prognostic importance of age (OR: 4.865), TBI use, the presence of aGVHD (OR: 2.794), CMV coinfection (OR: 2.261), and Cy use (OR: 27.353). A statistically significant difference was found between the mean BKV‐HC follow‐up times compared with post‐transplantation Cy intake (p <.001). The BKV‐HC rate increased as the number of risk factors of the patient increased. Conclusion: BKV‐HC is an essential complication of HSCT primarily associated with Cy use, the presence of aGVHD, and donor relatedness. The present study shows that the use of Cy in the post‐transplantation period further increases BKV‐HC risk in pediatric patients, regardless of dose. [ABSTRACT FROM AUTHOR]

Sirenomelia sequence is a rare congenital anomaly. This is also known as "mermaid syndrome" because of typical feature of lower limb. Sirenomelia sequence is characterized with a single midline lower limb. Our case was an infant delivered at 34 gestational weeks by spontaneous vaginal delivery from a 34 years old gravida 2, para 0. Parents are not relative. Infant has one femur, one tibia and one phalanx at lower extremity. Calcaneus, metatarsals and other bones of the foot are absent. Patient has anal atresia and renal agenesy. Determination of sex was impossible since external genitalia was absent. Although some risk factors (e.g. maternal diabetes) have been suggested, etiolgy of sirenomelia sequence is debated. In this report, we describe a premature infant with sirenomelia sequence because of very rare presentation. [ABSTRACT FROM AUTHOR]