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Öğe A comparison of hypersensitivity reactions between intravenous and intramuscular applications of native E. coli asparaginase in children with acute lymphoblastic leukemia(Sage Publications Ltd, 2023) Al, Isik Odaman; Ozdemir, Nihal; Ersoy, Gizem Zengin; Bayram, Cengiz; Cilengiroglu, Ozgul Vupa; Arslantas, Esra; Uysalol, Ezgi PasliIntroduction Asparaginase is an indispensable drug in treating childhood acute lymphoblastic leukemia (ALL). Hypersensitivity reactions (HSR) are the most common side effects and interfere with the antineoplastic activity of the drug. This study aims to compare the intramuscular (IM) and intravenous (IV) administration routes of Native Escherichia coli Lasparaginase (L-ASNase) in terms of hypersensitive reactions. Methods L-ASNase was randomly administered IV or IM to newly diagnosed ALL patients and HSR was monitored in all patients for 1 h following the end of the IV infusion and for 2 h following the end of the IM administration of L-ASNase. Based on a retrospective review of clinical charts, reactions were identified. In order to determine the severity of each allergic reaction, we used the Common Terminology Criteria for Adverse Events (CTCAE) version 4.03 for allergic reactions. Results A total of 1032 doses of L-ASNase were administered to 85 patients (42 males and 43 females) during the study period. Among 85 patients, 30 reactions were recorded, which means that 35% of the patients reacted. According to the CTCAE, twenty-nine out of 30 reactions (97%) were grade 2, while one (3%) was grade 4. In terms of individual doses, there was a non-significant trend toward increased incidence of reactions with IV administration (3.8% versus 0.9%, p = 0.064). The rate of reactions was higher in patients who received all IV doses (n: 60) as compared to those who received all IM doses (n: 25) (31.7% vs. 3.5%; chi-square= 8.415, p value=0.04). Based on the risk groups and HSR incidence, it was found that high risk group (HRG) patients were significantly more likely to develop HSR compared to the standart risk group (SRG) and intermediate risk group (MRG) patients (chi-square p = 0.003, CI: 95%; odds ratio: 3.12 and 5.91, respectively). Conclusions In conclusion, IM administration of L-ASNase causes significantly less HSR to L-ASNase than the IV route. Patients with HRGALL have a higher risk of HSR. Since L-ASNase is still used in many developing countries and there are problems in the supply of Erwinia chrysanthemi ASNase (Erwinia), LASNase can be administered IM to reduce the frequency of HSR.Öğe Hepatocellular Carcinoma in ADA-SCID Patient After Hematopoietic Stem Cell Transplantation(Lippincott Williams & Wilkins, 2023) Ucku, Duygu; Armutlu, Ayse; Cipe, Funda; Ersoy, Gizem Zengin; Karakaya, Afak Durur; Arikan, CigdemAdenosine deaminase (ADA) deficiency is one of the most prevalent forms of severe combined immunodeficiency and results in the accumulation of toxic substrates which creates a systemic metabolic disease. It predisposes patients to the development of malignancies, most commonly lymphoma. We report an 8-month-old infant with ADA deficient severe combined immunodeficiency who developed progressive liver dysfunction and hepatocellular carcinoma after successful hematopoietic stem cell transplantation. This is the first case report of an ADA-deficient patient who presented with hepatocellular carcinoma and gives an insight into the complex etiology that can lie behind liver dysfunction in these patients.Öğe THE IMPACT OF TREOSULFAN -BASED CONDITIONING FOR PRIMARY IMMUNE DEFICIENCIES: SINGLE CENTER EXPERIENCE(Springernature, 2023) Ersoy, Gizem Zengin; Cipe, Funda; Alsoy, Basak Adakli; Hashemi, Nazli; Oner, Ozlem Basoglu; Aydogdu, Selime; Dikme, Gurcan[Abstract Not Available]Öğe The impact of Treosulfan-based conditioning for inborn errors of immunity: Is dose monitoring crucial?(Wiley, 2023) Ersoy, Gizem Zengin; Cipe, Funda; Fisgin, Tunc; Aksoy, Basak Adakli; Oner, Ozlem Basoglu; Hashemi, Nazli; Aydogdu, SelimeIntroductionIn children with inborn errors of immunity (IEI) who will receive a hematopoietic stem cell transplant (HSCT) treosulfan-based conditioning is currently preferred. The aim of this study was to investigate early and late outcomes in pediatric IEI patients receiving pre-HSCT treosulfan and to examine the effect of treosulfan dose monitoring on outcomes. MethodsSeventy-three pediatric patients receiving this management between 2015 and 2022 were included. ResultsOverall survival rate was 80%, and event-free survival was 67.8%. A larger treosulfan dose AUC after first application increased the rate of early toxicity (p = .034) and slowed lymphocyte engraftment (r = .290; p = .030). Underlying disease, treosulfan AUC, donor type, stem cell type, number of immunosuppressive agents, the dose of anti-thymocyte globulin, and post-transplantation cyclophosphamide did not to increase risk of acute graft-versus-host disease. The risk of mixed chimerism (MC) in patients with autoimmune lymphoproliferative syndrome and leukocyte adhesion deficiency were higher than those with severe combined immunodeficiency (p = .021 and p = .014, respectively). The risk of MC was lower in those receiving peripheral blood stem cells (SC) compared with bone marrow derived SC (OR = .204, p = .022). ConclusionThe AUC of the treosulfan dose was not associated with poorer late outcomes. Treosulfan is an agent that can be used safely in the IEI patient group, level measurement appears essential to identify early toxicities. Prospective studies with more extended follow-up periods are needed.Öğe WOULD MONITORIZING TREOSULFAN LEVELS IN PATIENTS TRANSPLANTED FOR TRANSFUSION DEPENDENT THALASSEMIA BE BENEFICIAL IN TERMS OF CHIMERIZM? A SINGLE CENTER EXPERIENCE(Springernature, 2023) Aksoy, Basak Adakli; Ersoy, Gizem Zengin; Elsayed, Mariam; Oner, Ozlem Basoglu; Aydogdu, Selime; Dikme, Gurcan; Erdem, Melek[Abstract Not Available]
