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    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, Cigdem
    Adenosine 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.
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    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]
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    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, Selime
    IntroductionIn 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.