Thalassemia-free and graft-versus-host-free survival: outcomes of hematopoietic stem cell transplantation for thalassemia major, Turkish experience
Makaleler > Tam Makale · 2022
Yazarlar: M. Akif Yesilipek, Vedat Uygun, Alphan Kupesiz, Gulsun Karasu, Gulyuz Ozturk, MEHMET ERTEM, Ilgen Sasmaz, Hayriye Daloglu, ELİF GÜLER, Volkan Hazar, Tunc Fisgin, GÜLAY SEZGİN, Savaş KANSOY, BÜLENT BARIŞ KUŞKONMAZ, Burcu Akinci, Namik Ozbek, Elif Unal Ince, Seda Ozturkmen, FUNDA TAYFUN KÜPESİZ, Koray Yalcin, Sema Anak, Ceyhun Bozkurt, MUSA KARAKÜKCÜ, SERHAN KÜPELİ, Davut Albayrak, Haldun Oniz, Serap AKSOYLAR, FATMA VİSAL OKUR, CANAN ALBAYRAK, Fatma DEMİR YENİGÜRBÜZ, Ikbal Ok Bozkaya, Talia Ileri, Orhan Gursel, Barbaros Sahin Karagun, GÜLEN TÜYSÜZ KİNTRUP, Suna Celen, Murat Elli, Basak Adakli Aksoy, EBRU YILMAZ, Atila Tanyeli, Sule Turan Akyol, Zuhal Onder Sivi, Gülcihan ÖZEK, FAHRİYE DUYGU ÇETİNKAYA, İBRAHİM KARTAL, DİDEM ATAY, ARZU AKYAY, Ozlem Arman Bilir, Hasan Fatih Cakmakl, Emin Kurekci, Baris Malbora, SİNAN AKBAYRAM, Haci Ahmet Demir, Suar Caki Kilic, ADALET MERAL GÜNEŞ, EMİNE ZENGİN, Salih Ozmen, Ali Bulent Antmen
BONE MARROW TRANSPLANTATION, SCI Expanded İndekslerine Giren Dergi, Uluslararası, 2022, cilt 57, ss 760-767
DOI: 10.1038/s41409-022-01613-w
Özet
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.