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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; Aksoy, Başak; Çokluk, Mila N.; Dikme, Gürcan; Cipe, Funda; Bozkurt, CeyhunAllogeneic hematopoietic stem cell transplantation (HSCT) is the curative therapy for beta-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 < 0.05). Furthermore, in the second step of investigating overall survival (OS), age, gender and Pesaro risk group (I-II vs. III) showed a significant difference (p < 0.05). There was no significant difference in transplant-related mortality (TRM) between groups. Non-sibling related donor transplants are important for populations where consanguineous marriages are common. Transplant groups according to graft type had similar thalassemia-free survival (TFS) and OS when using a treosulfan-based regimen in our study.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; Aksoy, Başak; Savcı, Yunus Emre; Dikme, Gürcan; Cipe, Funda; Fışgın, Tunç[No abstract available]Öğe Donor lymphocyte infusion administrations after allogeneic stem cell transplantations in pediatrics: A single center experience(Nature Publishing Group, 2019) Aydoğdu, Selime; Mergen, Azize; Aksoy, Başak; Akbay, Hazal S.; Cipe, Funda; Dikme, Gürcan; Bozkurt, Ceyhun; Fışgın, TunçAllogeneic stem cell transplantation (allo-SCT) is considered the cornerstone in the treatment of several malignant and not malignant hematological diseases. However, relapse of hematological disease after allo-SCT is considered the most challenging point in the field. The risk can be reduced through optimal patients, donor and disease selection before allo-SCT, but harnessing donor immune system is an appealing way to treat or avoid disease relapse. Donor lymphocyte infusion (DLI) is a simple and effective therapy after allo-SCT. In this paper, the efficacy of DLI will be analyzed in different hematological diseases, focusing also on their therapeutic or pre-emptive use.Öğe Malignancies and lymphoproliferations in children with primary immune deficiency-a single-center experience(2024) Aydogmus, Cigdem; Turkyilmaz Ucar, Ozge; Kaplan Sarikavak, Sibel; Cipe, Funda; Ulas, Selami; Turan, Isilay; Naiboglu, Sezin; Yildirim, Ilke; Kalay, Gulsah; Kapci, Nermin; Al, Serdar; Gokmirza Ozdemir, Pinar; Celiksoy, Mehmet HalilPrimary immune deficiencies (PIDs) are rare genetic disorders characterized by impaired immune function, leading to frequent infections and immune dysregulation. Studies have shown that individuals with PID are at an increased risk of developing malignancies and lymphoproliferative disorders compared with the general population. In this single-center study, we aimed to analyze the occurrence of malignancies and lymphoproliferations in children diagnosed with PID. We retrospectively analyzed the medical records of 550 pediatric patients diagnosed with PIDs at our center. Among them, 17 (3,0%) patients were identified with malignancy and/or benign lymphoproliferation. Eight of the 17 patients (47.0%) had immune dysregulatory diseases, whereas ataxia-telangiectasia was the second most common PID associated with malignancy and/or benign lymphoproliferation (n = 5, 29.4%). Lymphoma was the predominant malignancy (n = 11, 64.7%), and Epstein-Barr virus was identified as the most common viral agent associated with malignancy and/or benign lymphoproliferation in patients with PID (n = 8, 47.0%). Our study highlights the association between PID and malignancies/lymphoproliferations, with immune dysregulation syndromes being the most common subclass associated with malignancies/lymphoproliferations. Early diagnosis, multidisciplinary management, and regular surveillance are crucial in improving patient outcomes and saving lives.