중증 재생불량빈혈 환아의 이식편 부전에서 2차 이식: 단일기관의 경험

중증 재생불량빈혈 환아의 이식편 부전에서 2차 이식: 단일기관의 경험

Second Allogeneic Stem Cell Transplantation for Graft Failure in children with Aplastic Anemia : A Single-Center Experience

(구연):
Release Date : 2013. 10. 19(토)
Hae In Jang, M.D., In Su Choi, M.D., Hee Jo Baek, M.D., Dong Kyun Han, M.D., Tai Ju Hwang, M.D., and Hoon Kook, M.D.
Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Gwangju, Korea
장해인, 최인수, 백희조, 한동균, 황태주, 국 훈
전남대학교 의과대학, 화순전남대학교병원 소아과학교실

Abstract

Background: Allogeneic hematopoietic stem cell transplantation (HSCT) has been accepted as a curative therapy for severe aplastic anemia (SAA). However, graft failure (GF) still remains a major problem, and a second allograft is a valuable option for these patients. Patients and Methods: From 1991 to 2013, 56 transplants were performed in 47 children with SAA at the Pediatric Blood & Marrow Transplantation Center, Chonnam National University. This study was a retrospective analysis of the medical records on 8 patients with SAA who received the 2nd HSCT after graft failure (GF). Results: Seven patients had acquired AA and one had Fanconi anemia. Among them, 7 underwent the 2nd HSCTs and the last patient underwent a 3rd syngeneic HSCT due to late GF. Stem cell sources for HSCT were matched sibling donors (n=4), syngeneic donors (n=2), and unrelated donors (n=2). All but 1 patient received stem cells from the original donor. One patient with Fanconi anemia was re-transplanted with matched unrelated bone marrow due to primary GF after 1 antigen-mismatched unrelated cord blood transplantation. Two patients who underwent unrelated HSCT developed grade I-II acute graft-versus-host disease. The Kaplan-Meier (K-M) 10-year overall survival after 2nd or 3rd HSCT was 100% with a median follow-up of 146 months (range, 13-186), and the 10-year K-M failure-free survival was 72.9%. Conclusion: Second HSCT for GF in SAA seems to be feasible and should be considered as a standard practice. However, further investigation in the best choice of 2nd transplant donor including haploidentical family donors, and refinement of conditioning regimens, especially in the syngeneic settings as well as the biological understanding of GF mechanism should be explored for those SAA patients who show GF after initial transplantation.

Keywords: Second Allogeneic Stem Cell Transplantation , ,