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    Are the anxiety levels of pediatric hematology-oncology patients different from healthy peers during the COVID-19 outbreak?
    (Lippincott Williams and Wilkins, 2020) Çakıroğlu, Süleyman; Yeltekin, Ceren; Fışgın, Tunç; Öner, Özlem B.; Aksoy, Başak A.; Bozkurt, Ceyhun
    The COVID-19 outbreak has caused anxiety among children with hematology-oncology disease and their families, as it has in every segment of society. In this study, we aimed to detect the anxiety levels of children with hematologic or oncologic disease and of their parents after the COVID-19 outbreak. The sample consisted of 15 patients aged 12 to 18 years receiving treatment in the Pediatric Hematology and Oncology Unit in Altinbaş University Medical Faculty Bahçelievler Medikalpark Hospital and 33 parents of the same unit patients aged between 6 and 18, and their 35 healthy peers and their parents. The State-Trait Anxiety Inventory was applied to participant children and their parents to evaluate their general anxiety and pandemic-related anxiety levels. Children with a hematology-oncology disease and their families were compared with healthy peers and their families. No significant difference was observed for pandemic-related anxiety levels (P>0.05). Both parent groups exhibited higher anxiety levels with regard to the pandemic than did their children (P<0.05). Children with hematology-oncology disease reported significantly higher trait anxiety levels when compared with healthy peers (P=0.01). The families of children who had not received stem cell transplantation had higher state and trait anxiety scores than the families of children who had received the transplantation (P<0.05). Even though they were in the high-risk group, children with a hematology-oncology disease and their families had pandemic-related anxiety levels comparable to those of healthy peers and their families. © 2020 Lippincott Williams and Wilkins. All rights reserved.
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    Biallelic form of a known CD3E mutation in a patient with severe combined immunodeficiency
    (Springer/Plenum Publishers, 2020) Erman, Baran; Fırtına, Sinem; Fışgın, Tunç; Bozkurt, Ceyhun; Cipe, Funda Erol
    To the Editor: T cell receptor (TCR) complex consists of αβ or γδ TCR chains in combination with four CD3 subunits, CD3ε, CD3γ, CD3δ, and CDζ [1]. This complex is required for thymocyte development and the initiation of T cell-mediated adaptive immune responses. Although TCR chains bind antigenic peptides presented by MHC molecules, the CD3 subunits provide transduction of signals into the cytosol for the activation and differentiation of T lymphocytes [2]. CD3 deficiencies can cause a rare form of severe combined immunodeficiency (SCID). Although CD3ε, CD3δ, and CDζ mutations usually result in a T- B+ +NK+ SCID phenotype, CD3γ deficiency leads to a milder phenotype with autoimmunity [3]. Only 2% of patients with SCID have TCR defects [3]. The T cell antigen receptor epsilon subunit (CD3E) gene is located at 11q23.3 and has been associated with autosomal recessive SCID [4]. Only a few mutations of the CD3E gene have been identified so far [4–8]. Here, we identified the biallelic form of a known CD3E mutation in a patient with a severe T- B+ NK+ phenotype.
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    CMV-specific T-Cells for treatment of CMV infection after hematopoietic stem cell transplantation in a pediatric case: first application in Turkey
    (2020) Celilova, Sevil; Toret, Ersin; Aksoy, Başak; Ovalı, Ercüment; Bozkurt, Ceyhun
    Cytomegalovirus (CMV) infection is still a major complication after allogeneic hematopoietic stem cell transplantation (HSCT) [1,2]. Unfortunately, prolonged antiviral treatment of CMV infection causes a delayed CMV-specific immune reconstitution. At this point, adoptive immunotherapy by CMV-specific T-cells can control CMV infection or provide immune reconstruction.
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    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, Ceyhun
    Allogeneic 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.
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    Comparison of hematopoietic stem cell transplantation results in patients with beta-thalassemia major from three different graft types
    (Taylor & Francis Ltd, 2021) Aydoğdu, Selime; Toret, Ersin; Aksoy, Başak A.; Aydın, Muhammed Fatih; Cipe, Funda E.; Bozkurt, Ceyhun; Fışgın, Tunç
    Allogeneic 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.
  • Küçük Resim
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    Could the COVID-19 infection have a better prognosis than expected in pediatric hematology oncology and bone marrow transplant patients?
    (Scientific Letter, 2021) Öner, Özlem Başoğlu; Aksoy, Barış Adaklı; Sütçü, Murat; Çipe, Funda; Atça, Ali Önder; Bozkurt, Ceyhun; Fışgın, Tunç
    Coronavirus disease 2019 (COVID-19) is a pandemic that spread rapidly worldwide (1). So far, very few reports concerning the impact of COVID-19 among patients with pediatric hematologic-oncologic diseases are available (2). We aimed to describe the clinical features, prevalence, treatments, and outcomes in the COVID-19 patient population.
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    COVID-19 disease in children and adolescents following allogeneic hematopoietic stem cell transplantation: A report from the Turkish pediatric bone marrow transplantation study group
    (2024) Bozkurt, Ceyhun; Hazar, Volkan; Malbora, Barış; Küpesiz, Alphan; Aygüneş, Utku; Fışgın, Tunç; Karakükçü, Musa; Kuşkonmaz, Barış; Kılıç, Suar Çakı; Bayırlı, Derya; Bilir, Özlem Arman; Yalçın, Koray; Gözmen, Salih; Uygun, Vedat; Elli, Murat; Sarbay, Hakan; Küpesiz, Funda Tayfun; Şaşmaz, Hatice İlgen; Aksoy, Başak Adaklı; Yılmaz, Ebru; Okur, Fatma Visal; Tekkeşin, Funda; Yenigürbüz, Fatma Demir; Özek, Gülcihan; Atay, Abdullah Avni; Bozkaya, İkbal Ok; Çelen, Suna; Öztürkmen, Seda; Güneş, Adalet Meral; Gürsel, Orhan; Güler, Elif; Özcan, Alper; Çetinkaya, Duygu Uçkan; Aydoğdu, Selime; Özbek, Namık Yaşar; Karasu, Gülsün; Sezgin, Gülay; Doğru, Ömer; Albayrak, Davut; Öztürk, Gülyüz; Aksoylar, Serap; Daloğlu, Hayriye; Al, Işık Odaman; Evim, Melike Sezgin; Akbayram, Sinan; Öncül, Yurday; Zengin, Emine; Albayrak, Canan; Timur, Çetin; Kar, Yeter Düzenli; Çakmaklı, Hasan Fatih; Tüfekçi, Özlem; Töret, Ersin; Antmen, Bülent
    Background: Data on the risk factors and outcomes for pediatric patients with SARS-CoV-2 infection (COVID-19) following hematopoietic stem cell transplantation (HSCT) are limited. Objectives: The study aimed to analyze the clinical signs, risk factors, and outcomes for ICU admission and mortality in a large pediatric cohort who underwent allogeneic HSCT prior to COVID-19 infection. Method: In this nationwide study, we retrospectively reviewed the data of 184 pediatric HSCT recipients who had COVID-19 between March 2020 and August 2022. Results: The median time from HSCT to COVID-19 infection was 209.0 days (IQR, 111.7-340.8; range, 0-3845 days). The most common clinical manifestation was fever (58.7%). While most patients (78.8%) had asymptomatic/mild disease, the disease severity was moderate in 9.2% and severe and critical in 4.4% and 7.6%, respectively. The overall mortality was 10.9% (n: 20). Deaths were attributable to COVID-19 in nine (4.9%) patients. Multivariate analysis revealed that lower respiratory tract disease (LRTD) (OR, 23.20, p: .001) and lymphopenia at diagnosis (OR, 5.21, p: .006) were risk factors for ICU admission and that HSCT from a mismatched donor (OR, 54.04, p: .028), multisystem inflammatory syndrome in children (MIS-C) (OR, 31.07, p: .003), and LRTD (OR, 10.11, p: .035) were associated with a higher risk for COVID-19-related mortality. Conclusion: While COVID-19 is mostly asymptomatic or mild in pediatric transplant recipients, it can cause ICU admission in those with LRTD or lymphopenia at diagnosis and may be more fatal in those who are transplanted from a mismatched donor and those who develop MIS-C or LRTD.
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    COVID-19 infection in children with cancer and stem cell transplant recipients in Turkey: A nationwide study
    (Wiley, 2021) Kebudi, Rejin; Kurucu, Nilgün; Tuğcu, Deniz; Hacısalihoğlu, Sadan; Fışgın, Tunç; Ocak, Süheyla; Kara, Ateş; Bozkurt, Ceyhun; Ince, Dilek; Aras, Seda; Aycicek, Ali; Aksoy, Basak Adakli; Karadas, Nihal; Ozturk, Gulyuz; Orhan, Mehmet Fatih; Ataseven, Eda; Akbayram, Sinan; Yilmaz, Ebru; Tufekci, Ozlem; Vural, Sema; Akyay, Arzu; Ayhan, Aylin Canbolat; Kilic, Suar; Uzel, Veysiye Hulya; Duzenli, Yeter; Acipayam, Can; Elli, Murat; Tanyeli, Atilla; Karakas, Zeynep; Somer, Ayper; Kara, Ates
    Adults with cancer are reported to have a higher risk for coronavirus disease (COVID-19) infection and more severe disease and mortality than the general population.1,2 Although children seem to be at a lower risk for COVID-19 than adults,3–5 data specifically addressing children with cancer are limited.6–12 We conducted a retrospective, multicenter, cross-sectional study on behalf of the Turkish Pediatric Hematology Society (TPHD) and Turkish Pediatric Oncology Group (TPOG) Society to analyze the characteristics of COVID-19 in all patients with cancer and stem cell transplant (SCT) recipients in all centers in Turkey, during March 11-May 31, 2020. Approval for the study was obtained by Turkish Ministry of Health (MoH), Istanbul University COVID Scientific Research Committee, and Istanbul University Ethics Committee. The study was carried out through the analysis of a questionnaire with 62 questions, which was sent to all members of the TPOG and TPHD Societies working in all 66 pediatric hematology/oncology departments in university, state, and private hospitals in Turkey. All replied and 53 patients were reported from the 24 centers.
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    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, Tunç
    Allogeneic 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.
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    Epidemiologic and microbiologic evaluation of catheter-line bloodstream infection in a pediatric hematopoietic stem cell transplant center
    (2023) Adaklı Aksoy, Başak; Kara, Manolya; Sütçü, Murat; Özbek, Ahmet; Zengin Ersoy, Gizem; Başoğlu Öner, Özlem; Aydoğdu, Selime; Gül, Doruk; Bozkurt, Ceyhun; Fışgın, Tunç
    Background: Children who underwent hematopoietic stem cell transplant (HSCT) are at high risk of developing central-line-associated bloodstream infection (CLABSIs). The present study aimed to identify possible risk factors for mortality by analyzing the clinical and laboratory characteristics of patients diagnosed with CLABSI in our pediatric HSCT unit. Methods: The initial CLABSI episodes of 102 children were analyzed. Medical records of the patients were evaluated by preformed standardized surveys. Univariate analysis and multivariate logistic regression analysis were performed to identify risk factors for mortality. Results: Thirty-five patients (34.3%) were female. The median age was 48 month (3-204). The median time to onset of CLABSI was 19 days (4-150). The Gram-negative/Gram-positive bacteria ratio among the causative agents was 57.8 % to 34.3 %. The mortality rate was 12.6%. The presence of severe neutropenia, initiation of inappropriate empirical antibiotic therapy, the presence of hypotension, persistent bacteremia, pediatric intensive care unit admission, growth of carbapenemase-positive Gram-negative microorganism and multi-drug resistant bacteria were significantly high in the mortality group when compared to survivors. The presence of hypotension, inappropriate empirical antibiotic therapy, and persistent bacteremia were found to be independent risk factors for mortality. Conclusion: Rational use of antibiotics, active surveillance/ screening of patients together with improved infection control practices may reduce the incidence and the consequences of CLABSIs.
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    Evaluation of the risk factors for BK virus-associated hemorrhagic cystitis in pediatric bone marrow transplantation patients: Does post-transplantation cyclophosphamide increase the frequency?
    (2022) Ersoy, Gizem Zengin; Bozkurt, Ceyhun; Aksoy, Başak Adaklı; Öner, Özlem Başoğlu; Aydoğdu, Selime; Çipe, Funda; Sütçü, Murat; Özkaya, Ozan; Fışgın, Tunç
    Background: 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.
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    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, Tunç
    [No abstract available]
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    Immune reconstitution of thalassemia major patients after 1 year of hematopoetic stem cell transplantation
    (Nature Publishing Group, 2019) Cipe, Funda Erol; Bozkurt, Ceyhun; Aksoy, Başak Adaklı; Aydoğdu, Selime; Dikme, Gürcan; Fışgın, Tunç
    Immune reconstitution inflammatory syndrome (IRIS) is a clinical condition emerging after immune recovery of an immunocompromised status, mostly in human immunodeficiency virus infected patients but also in several other settings, such as the recovery from the severe combined immunodeficiency status after hematopoietic stem cell transplantation. Herein, we report a patient transplanted for severe combined immunodeficiency who developed IRIS for 2 times, namely shortly after transplantation and after donor lymphocyte infusion. Pediatric transplant teams need to be aware of the previous IRIS phenomenon of BCG-adenitis while making the decision of donor lymphocyte infusions.
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    Langerhans Hücreli Histiositoz: Tek Merkez Deneyimi
    (2022) Bilgin, Burçak Kurucu; Yeşil, Süleyman; Bozkurt, Ceyhun; Yüksek, Nazmiye; Fettah, Ali; Şahin, Gürses
    Amaç: Langerhans hücreli histiositoz (LHH), tüm organ ve sistemleri etkileyebilen ve çok çeşitli klinik bulgulara neden olabilen nadir bir hastalıktır. Tedavi ve prognoz organ tutulumu bölgesine ve riskine göre değişkenlik gösterir. Bu çalışmada, kliniğimizde LHH tanısıyla takip edilen hastaları değerlendirmeyi amaçladık. Gereç ve Yöntemler: Çalışmada Ocak 2000 ile Aralık 2019 arasında LCH tanısı alan 24 hastanın verileri geriye dönük olarak incelendi. Hastalar tanı anındaki yaş, cinsiyet, başvuru semptomları, organ tutulumu, tedaviler, takip süresi ve tetavi yanıtları açısından değerlendirildi. Bulgular: Hastaların tanı yaşı ortalaması 4,7±4,6 (0,6-16,6) yıl ve ortalama takip süresi 91,6±67,8 (12,1-240) ay idi. Tanı anındaki en sık görülen bulgu cilt lezyonları iken, en sık görülen organ tutulumları kemik ve ciltti. Bunu sırasıyla hipofiz, karaciğer, lenf nodu, akciğer, dalak, periodontal bölge, tiroid, orbital bölge, mastoid, pankreas ve kemik iliği izledi. Hastalara risk gruplarına göre LCH-III çalışma protokolüne uygun olarak tedavi uygulandı. Progresyon gösteren 6 hastanın 5’ine kladribin tedavisi, 1 hastaya hematopoetik kök hücre nakli (HKHN) uygulandı ve hepsinde tam yanıt elde edildi. Sonuç: LHH’de tutulan organ ve sistemlere, hastalığın yaygınlığına göre kemoterapi, radyoterapi ve cerrahi tedavi seçenekleri farklı kombinasyonlarda uygulanmaktadır. Yanıtsızlık veya progresyon durumlarında bizim serimizde olduğu gibi kladribin ve HKHN tedavileri başarılı bir şekilde uygulanmaktadır.
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    Neurologic status of patients with purine nucleoside phosphorylase deficiency before and after hematopoetic stem cell transplantation
    (2023) Gemici Karaaslan, Betül; Turan, Işılay; Aydemir, Sezin; Akyüncü Meriç, Zeynep; Atay, Didem; Akçay, Arzu; Ayaz Sarı, Aysun; Hersfield, Michael; Çipe, Funda; Adaklı Aksoy, Başak; Zengin Ersoy, Gizem; Bozkurt, Ceyhun; Kendir Demirkol, Yasemin; Öztürk, Gülyüz; Aydoğmuş, Çiğdem; Kıykım, Ayça; Çokuğraş, Haluk
    Background Purine nucleoside phosphorylase (PNP) defciency is a rare autosomal recessive combined immunodefciency. The phenotype is profound T cell defciency with variable B and NK cell functions and results in recurrent and persistent infections that typically begin in the frst year of life. Neurologic fndings occur in approximately two-thirds of patients. The mechanism of neurologic abnormalities is unclear. Hematopoietic stem cell transplantation (HSCT) is the only curative treatment for PNP defciency. Methods We report here six patients from fve unrelated families with PNP defciency treated in two centers in Turkey. We evaluated the neurological status of patients and compared to post-transplantation period if available. Then, we performed PubMed, Google Scholar, and Researchgate searches using the terms “PNP” and “hematopoietic stem cell transplantation” to fnd all reported cases of PNP transplantation and compared to our cohort. Results Six patients were treated in two centers in Turkey. One patient died from post-transplant complications. The other four patients underwent successful HSCT with good immune reconstitution after transplantation (follow-up 21–48 months) and good neurological outcomes. The other patient with a new mutation is still waiting for a matching HLA donor. Discussion In PNP defciency, clinical manifestations are variable, and this disease should be considered in the presence of many diferent clinical fndings. Despite the comorbidities that occurred before transplantation, HSCT currently appears to be the only treatment option for this disease. HSCT not only cures immunologic disorders, but probably also improves or at least stabilizes the neurologic status of patients.
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    Outcomes of hematopoietic stem cell transplantation in patients with thalassemia major: how do anti-HLA antibodies impact?
    (Wiley, 2024) Ersoy, Gizem Zengin; Aksoy, Başak Adaklı; Erdem, Melek; Karataş, Lokman; Aydoğdu, Selime; Öner, Özlem Başoğlu; Dikme, Guercan; Bozkurt, Ceyhun; Fışgın, Tunç
    AimTo 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).MethodsOne 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).ResultsThe 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.ConclusionAnti-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.
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    Outcomes of hematopoietic stem cell transplantation in patients with thalassemia major: how do anti-HLA antibodies impact?: the impact of anti-HLA antibodies on transplantation outcomes in thalassemia major
    (2024) Ersoy, Gizem Zengin; Aksoy, Başak Adaklı; Erdem, Melek; Karataş, Lokman; Aydoğdu, Selime; Başoğlu Öner, Özlem; Dikme, Gürcan; Bozkurt, Ceyhun; Fışgın, Tunç
    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.
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    Primary immunodeficiencies: HSCT experiences of a single center in Turkey
    (Pediatric Transplantation, 2021) Cipe, Funda Erol; Aydoğdu, Selime; Dikme, Gürcan; Kıykım, Ayça; Aydoğmuş, Çiğdem; Yücel, Esra; Bozkurt, Ceyhun; Fışgın, Tunç
    Background 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.
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    Tandem high-dose chemotherapy followed by autologous stem cell transplantation: An infant with trilateral retinoblastoma
    (2023) Töret, Ersin; Özdemir, Zeynep Canan; Zengin Ersoy, Gizem; Öztunalı, Çiğdem; Bozkurt, Ceyhun; Kebudi, Rejin
    Background: Retinoblastoma (RB) is the most common intraocular malignancy in childhood. Advanced RB, associated with exceedingly poor prognosis, requires more intensive multiagent chemotherapy than conventional regimens. Rescue of the bone marrow after intensive chemotherapy is achieved with stem cell transplantation. The sequential courses (tandem transplantation) of high-dose chemotherapy followed by autologous stem cell transplantation allow for even greater dose intensity in consolidation with the potential to use different active chemotherapeutics at each transplant and have proven feasible and successful in treating children with recurrent/refractory solid tumors. Case description: We report an infant with trilateral high-risk RB who received tandem high-dose chemotherapy (HDC) followed by autologous stem cell transplantation after the conventional chemotherapy. A 5-month-old female patient presented with strabismus, and the ophthalmoscopic examination showed intraocular tumoral lesions in both eyes. Magnetic resonance imaging (MRI) concluded the trilateral retinoblastoma diagnosis due to a tumoral mass in the optic chiasm. The follow-up ophthalmologic examinations and the MRI detected stable disease after six cycles of multiagent chemotherapy. Conclusions: Rescue with autologous stem cell transplantation after HDC allows for an increase in chemotherapy intensity. Tandem transplantation provides the chance to perform different chemotherapeutics at each transplant and enables an increase in the chemotherapy intensity, thus providing a positive effect on disease-free survival.
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    Öğe
    Thalassemia-free and graft-versus-host-free survival: Outcomes of hematopoietic stem cell transplantation for thalassemia major, Turkish experience
    (Bone Marrow Transplantation, 2022) Yeşilipek, M. Akif; Uygun, Vedat; Küpesiz, Alphan; Karasu, Gülsün; Öztürk, Gülyüz; Ertem, Mehmet; Şaşmaz, İlgen; Daloğlu, Hayriye; Güler, Elif; Hazar, Volkan; Fışgın, Tunç; Sezgin, Gülay; Kansoy, Savaş; Kuşkonmaz, Barış; Akıncı, Burcu; Özbek, Namık; Ünal-İnce, Elif; Öztürkmen, Seda; Küpesiz-Tayfun, Funda; Yalçın, Koray; Anak, Sema; Bozkurt, Ceyhun; Karakükçü, Musa; Küpeli, Serhan; Albayrak, Davut; Öniz, Haldun; Aksoylar, Serap; Visal-Okur, Fatma; Albayrak, Canan; Demir-Yenigürbüz, Fatma; Ok-Bozkaya, İkbal; İleri, Talia; Gürsel, Orhan; Karagün, Barbaros Şahin; Tüysüz-Kintrup, Gülen; Çelen, Suna; Elli, Murat; Adaklı-Aksoy, Başak; Yılmaz, Ebru; Tanyeli, Atila; Turan-Akyol, Şule; Önder-Siviş, Zuhal; Özek, Gülcihan; Uçkan, Duygu; Kartal, İbrahim; Atay, Didem; Arzu, Akyay; Arman-Bilir, Özlem; Çakmaklı, Hasan Fatih; Kürekçi, Emin; Malbora, Barış; Akbayram, Sinan; Demir, Hacı Ahmet; Kılıç, Suar Çakı; Güneş, Adalet Meral; Zengin, Emine; Özmen, Salih; Antmen, Ali Bülent
    We report the national data on the outcomes of hematopoietic stem cell transplantation (HSCT) for thalassemia major (TM) patients in Turkey on behalf of the Turkish Pediatric Stem Cell Transplantation Group. We retrospectively enrolled 1469 patients with TM who underwent their first HSCT between 1988 and 2020 in 25 pediatric centers in Turkey. The median follow-up duration and transplant ages were 62 months and 7 years, respectively; 113 patients had chronic graft versus host disease (cGVHD) and the cGVHD rate was 8.3% in surviving patients. Upon the last visit, 30 patients still had cGvHD (2.2%). The 5-year overall survival (OS), thalassemia-free survival (TFS) and thalassemia-GVHD-free survival (TGFS) rates were 92.3%, 82.1%, and 80.8%, respectively. cGVHD incidence was significantly lower in the mixed chimerism (MC) group compared to the complete chimerism (CC) group (p < 0.001). In survival analysis, OS, TFS, and TGFS rates were significantly higher for transplants after 2010. TFS and TGFS rates were better for patients under 7 years and at centers that had performed over 100 thalassemia transplants. Transplants from matched unrelated donors had significantly higher TFS rates. We recommend HSCT before 7 years old in thalassemia patients who have a matched donor for improved outcomes.