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Öğe Assessment of serum vitamin D in pediatric hematopoietic stem cell transplantation patients and its relation with complications(Nature Publishing Group, 2018) Karakaş, Zeynep; Aydoğdu, Selime; Yılmaz, Yasin; Bozkurt, Ceyhun; Aykanat, Alper; Fışgın, TunçRecently, evaluation of serum vitamin D level in pediatric hematopoietic stem cell transplantation (HSCT) patients gained popularity, as there is close relation with development of GVHD and complications. Our aim was to assess the serum vitamin D level and its relation with GVHD and complications such as graft failure in HSCT patients.Öğe Comparison of allogeneic stem cell transplantation results from related or unrelated donors in beta-thalassemia major(Nature Publishing Group, 2019) Aydoğdu, Selime; Mergen, Azize; Adaklı Aksoy, Başak; Çokluk, Mila N.; Dikme, Gürcan; Erol Cipe, Funda; Fışgın, Tunç; Bozkurt, CeyhunAlthough there have been significant improvements with conventional therapies in beta thalassemia major, hematopoietic stem cell transplantation is only curative therapy. Related donors are preferred to diminish transplant risks. In lack of identical related donor, identical unrelated donors are second best choice. In this study, thalassemia major patients transplanted from unrelated donors (MUD) were compared with thalassemic patients transplanted from relative donor (MRD) retrospectively.Öğe Comparison of hematopoietic stem cell transplantation results in patients with ?-thalassemia major from three different graft types(Taylor & Francis Online, 2021) Aydoğdu, Selime; Töret, Ersin; Adaklı Aksoy, Başak; Aydın, Muhammed Fatih; Erol Cipe, Funda; Bozkurt, Ceyhun; Fışgın, TunçAllogeneic hematopoietic stem cell transplantation (HSCT) is the curative therapy for ?-thalassemias that induces severe life-threatening complications. The human leukocyte antigen (HLA) registries and umbilical cord blood banks have carried out diligent searches to find matched unrelated donors (MUDs) for about 70.0% of patients from 2000 onwards. The chance of finding a non-sibling fully matched family donors is higher in some ethnic groups in which consanguineous marriages are common. We have studied and compared transplant complications and outcomes in different graft types (sibling, non-sibling family and unrelated). The non-sibling matched family donor (MFD) group consisted of four mothers, three fathers, five cousins, one paternal uncle and one paternal aunt. There was no significant difference in the mean transfused CD34+ cells, engraftment, median days of neutrophil and platelet recovery were achieved (p?>?0.05). The distribution of postttransplant complication did not show any significant difference between groups (p?>?0.05). In univariate analysis and multivarite analyses, age, gender, Pesaro risk group (I-II vs. III) and ABO incompatibilty demonstrated a significant difference in disease free survival (p?Öğe Comparison of turkish stem cell coordination center (TURKOK) with Istanbul university bone marrow bank (TRIS); A single center experience in match unrelated donors(Nature Publishing Group, 2019) Mergen, Azize; Aydoğdu, Selime; Adaklı Aksoy, Başak; Savcı, Yunus Emre; Dikme, Gürcan; Erol Cipe, Funda; Bozkurt, Ceyhun; Fışgın, TunçMatch family donors are the preferable options in allogenic stem cell transplant. However, in the absence of donor relatives match unrelated donors have been an option. In this study, the donor screening, transplant preparation phases of Turkish Stem Cell Coordination Center (TURKOK) and the İstanbul University Bone Marrow Bank (TRIS), were compared.Öğe Donor lymphocyte infusion administrations after allogeneic stem cell transplantations in pediatrics: a single center experience(Nature Publishing Group, 2019) Aydoğdu, Selime; Mergen, Azize; Adaklı Aksoy, Başak; Akbay, Hazal S.; Erol Cipe, Funda; Dikme, Gürcan; Fışgın, Tunç; Bozkurt, CeyhunLoss of chimerism is one of the major problems after allogeneic stem cell transplantation(SCT). Donor- lymphocyte infusions(DLI) are used as a treatment after taper or stopping immunosuppression. In this study, DLI experience in 20 patients with loss of chimerism after SCT due to various benign and malign hematological diseases was presented.Öğe Evaluation of the risk factors for BK virus-associated hemorrhagic cystitis in pediatric bone marrow transplantation patients: Does post-transplantation cyclophosphamide increase the frequency?(Wiley, 2022) Zengin Ersoy, Gizem; Bozkurt, Ceyhun; Adaklı Aksoy, Başak; Başoğlu Öner, Özlem; Aydoğdu, Selime; Çipe, Funda; Sütçü, Murat; Özkaya, Ozan; Tunç, FışgınBackground: BKV-HC is one of the most significant complications of HSCT. This ret -rospective 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.Öğe Extracorporeal photopheresis treatment for steroid resistant graft versus host disease in pediatrics: single center experience(Nature Publishing Group, 2019) Aksoy, Başak Adaklı; Savcı, Yunus Emre; Mergen, Azize; Aydoğdu, Selime; Dikme, Gürcan; Bozkurt, Ceyhun; Fışgın, TunçBackground: Steroid resistant graft vs host disease (GVHD) is de?ned as unresolved or worsened symptomatology of GVHD at day 14 despite effective immunosuppression with steroid therapy. Second line immunosuppressive agents, extracorporeal photopheresis (ECP) and/or mesenchymal stem cell therapies are being tried as salvage therapy.Öğe Hit three birds with one stone: successful stem cell transplantation from one family donor to three siblings(Nature Publishing Group, 2019) Aksoy, Başak Adaklı; Aydoğdu, Selime; Dikme, Gürcan; Mergen, Azize; Bozkurt, Ceyhun; Fışgın, TunçBackground: Thalassemia major is a single gene related hereditary hemoglobinopaty required life long regular transfusions with chelation therapies. Stem cell transplantation is the only curative treatment of the disease. Three sibling thalassemia patients 13,4 and 3 years old were succesfully transplanted from their only donor,fully matched seventeen years old sister at our center.Öğe Invasive saprochaete capitata Infection in a patient with autosomal recessive CARD9 deficiency and a review of the literature(Springer/Plenum Publishers, 2020) Erman, Baran; Fırtına, Sinem; Aksoy, Başak Adaklı; Aydoğdu, Selime; Genç, Gonca Erköse; Doğan, Öner; Bozkurt, Ceyhun; Fışgın, Tunç; Erol Çipe, FundaPurpose Autosomal recessive (AR) CARD9 deficiency is an inherited immune disorder which results in impaired innate immunity against various fungi. Superficial and invasive fungal infections, mainly caused by Candida or Trichophyton species, are the hallmark of CARD9 deficiency. Together with the increasing number of CARD9-deficient patients reported, different pathogenic fungal species have been described such as Phialophora, Exophiala, Corynespora, Aureobasidium, and Ochroconis. Saprochaete capitata is an opportunistic infectious agent in immunocompromised patients and is a common cause of invasive fungal disease in patients with hematological malignancies. In this study, we investigated the causative genetic defect in a patient with S. capitata fungal infection which disseminated to lymph nodes and common bile duct. Methods The identification of the isolated yeast strain was made by direct microscopic examination and confirmed by internal transcribed spacer (ITS) sequencing. We applied whole exome sequencing to search for the disease-causing mutation. Sanger sequencing was used to validate the mutation in the patient and his parents. Results S. capitata was isolated from the biopsy specimen as the causative microorganism responsible for the invasive fungal disease in the patient. Whole exome sequencing revealed a homozygous c.883C > T, (p.Q295*) mutation in CARD9, confirmed by Sanger sequencing. Conclusions This is the first report of invasive Saprochaete infection associated with autosomal recessive (AR) CARD9 deficiency in the literature and thereby further extends the spectrum of fungal diseases seen in these patients.Öğe Primary immunodeficiencies: HSCT experiences of a single center in Turkey(WILEY, 2021) Erol Çipe, Funda; Adaklı Aksoy, Başak; Aydoğdu, Selime; Dikme, Gürcan; Kıykım, Ayca; Aydoğmus, Çiğdem; Yücel, Esra; Bozkurt, Ceyhun; Fışgın, TunçBackground Primary immunodeficiency diseases (PID) are characterized by the occurrence of frequent infections and are caused by many genetic defects. Hematopoietic stem cell transplantation (HSCT) is the only curative treatment option for the majority of PID. As a Pediatric Hematology-Oncology-Immunology Transplantation Unit, we wanted to present our HSCT experience regarding treatment of primary immunodeficiency diseases. Methods 58 patients were included in the study between January 2014 and June 2019. We searched 9/10 or 10/10 matched-related donor (MRD) firstly, in the absence of fully matched-related donor. We screened matched unrelated donor (MUD) from donor banks. MRD was used in 24 (41.3%) patients, MUD in 20 (34.4%) patients, and haploidentical donors in 14 (24.1%) patients. Demographic data, HSCT characteristics, and outcome were evaluated. While 16 patients had severe combined immunodeficiency (SCID), the remaining was non-SCID. Results Of the 58 patients, 38 were male and 20 were female. Median age at transplantation was 12 months (range: 2.5-172 months). Combined immunodeficiencies consisted 67.2% of patients. Mean follow-up time was 27 months (6 months-5 years). Median neutrophil, lymphocyte, and thrombocyte engraftment days were similar in comparison of both donor type and stem cell source. The most common complication was acute GvHD in 15 (25.8%) patients. In total, five patients (31%) belonging to the SCID group and 10 patients (23.8%) belonging to the non-SCID group died. Our total mortality rate was 15 (25.8%) in all patients. Conclusions We would like to present our HSCT experiences as a pediatric immunology transplantation center. Existing severe infections before transplantation period, BCGitis, and CMV are important issues of transplantation in Turkey. However, the follow-up time is shorter than some studies, our results regarding complications and survival are similar to previous reports.Öğe The use of allogeneic mesenchymal stem cells in childhood steroid-resistant acute graft-versus-host disease: a retrospective study of a single-center experience(Galenos Yayincilik, 2019) Bozkurt, Ceyhun; Karaöz, Erdal; Adaklı Aksoy, Başak; Aydoğdu, Selime; Fışgın, TunçObjective: 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.
