Thalassemia-free and graft-versus-host-free survival: outcomes of hematopoietic stem cell transplantation for thalassemia major, Turkish experience
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Tarih
2022
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info:eu-repo/semantics/closedAccess
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.
Açıklama
Anahtar Kelimeler
Kaynak
Bone Marrow Transplant
WoS Q Değeri
Q1
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Yesilipek MA, Uygun V, Kupesiz A, Karasu G, Ozturk G, Ertem M, Şaşmaz İ, Daloğlu H, Güler E, Hazar V, Fisgin T, Sezgin G, Kansoy S, Kuşkonmaz B, Akıncı B, Özbek N, İnce EÜ, Öztürkmen S, Küpesiz FT, Yalçın K, Anak S, Bozkurt C, Karakükçü M, Küpeli S, Albayrak D, Öniz H, Aksoylar S, Okur FV, Albayrak C, Yenigürbüz FD, Bozkaya İO, İleri T, Gürsel O, Karagün BŞ, Kintrup GT, Çelen S, Elli M, Aksoy BA, Yılmaz E, Tanyeli A, Akyol ŞT, Siviş ZÖ, Özek G, Uçkan D, Kartal İ, Atay D, Akyay A, Bilir ÖA, Çakmaklı HF, Kürekçi E, Malbora B, Akbayram S, Demir HA, Kılıç SÇ, Güneş AM, Zengin E, Özmen S, Antmen AB. Thalassemia-free and graft-versus-host-free survival: outcomes of hematopoietic stem cell transplantation for thalassemia major, Turkish experience. Bone Marrow Transplant. 2022 Feb 24.
