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Öğe Different kinetics and risk factors for ısolated extramedullary relapse after allogeneic hematopoietic stem cell transplantation in children with acute leukemia(Elsevier, 2021) Hazar, Volkan; Öztürk, Gülyüz; Yalçın, Koray; Uygun, Vedat; Aksoylar, Serap; Küpesiz, A.; Ok Bozkaya, İkbal; Karagün, Barbaros Şahin; İleri, Talia; Atay, Didem; Koçak, Ülker; Tezcan Karasu, Gülsün; Yeşilipek, Akif; Gökçe, Müge; Kansoy, Savaş; Tüysüz Kintrup, Gülen; Karakükçü, Musa; Visal Okur, Fatma; Ertem, Mehmet; Kaya, Zühre; Gürsel, Orhan; Yaman, Yöntem; Özbek, Namık; Antmen, Bülent; Tüfekçi, Özlem; Albayrak, Canan; Adaklı Aksoy, Başak; Sezgin, Gülay; Albayrak, Davut; Sezgin Evim, Melike; Zengin, Emine; Pekpak, Esra; Turkish Pediatric Bone Marrow Transplantation Study GroupRelapse after allogeneic hematopoietic stem cell transplantation (allo-HSCT) remains the most frequent cause ofpost-transplantation mortality. Isolated extramedullary (EM) relapse (iEMR) after HSCT is relatively rare and notwell characterized, particularly in pediatric patients. We retrospectively analyzed 1527 consecutive pediatricpatients with acute leukemia after allo-HSCT to study the incidence, risk factors, and outcome of iEMR comparedwith systemic relapse. The 5-year cumulative incidence of systemic relapse (either bone marrow [BM] only or BMcombined with EMR) was 24.8%, and that of iEMR was 5.5%. The onset of relapse after allo-HSCT was significantlylonger in EM sites than in BM sites (7.19 and 5.58 months, respectively;P= .013). Complete response (CR) 2+/active disease at transplantation (hazard ratio [HR], 3.1;P<.001) and prior EM disease (HR, 2.3;P= .007) were independent risk factors for iEMR. Chronic graft-versus-host disease reduced the risk of systemic relapse (HR, 0.5;P= .043) but did not protect against iEMR. The prognosis of patients who developed iEMR remained poor but wasslightly better than that of patients who developed systemic relapse (3-year overall survival, 16.5% versus 15.3%;P= .089). Patients experiencing theirfirst systemic relapse continued to have further systemic relapse, but only aminority progressed to iEMR, whereas those experiencing their iEMR atfirst relapse developed further systemicrelapse and iEMR at approximately similar frequencies. A second iEMR was more common after afirst iEMR thanafter afirst systemic relapse (58.8% versus 13.0%;P= .001) and was associated with poor outcome. iEMR has apoor prognosis, particularly after a second relapse, and effective strategies are needed to improve outcomes.© 2021 The American Society for Transplantation and Cellular Therapy. Published by Elsevier Inc. All rights reserved.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 BülentWe 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.