Favorable outcomes with durable chimerism after hematopoietic cell transplantation using busulfan and fludarabine-based reduced-intensity conditioning for pediatric patients with hemophagocytic lymphohistiocytosis

Jin Kyung Suh; Young Kwon Koh; Sung Han Kang; Hyery Kim; Eun Seok Choi; Kyung-Nam Koh; Ho Joon Im

doi:10.5045/br.2022.2022047

추천

검색

자료유형: 학술저널

저자정보: Jin Kyung Suh (Department of Pediatrics Korea Cancer Center Hospital Korea Institute of Radiological) Young Kwon Koh (Division of Pediatric Hematology/Oncology Department of Pediatrics Asan Medical Center Children’s H) Sung Han Kang (Division of Pediatric Hematology/Oncology Department of Pediatrics Asan Medical Center Children’s H) Hyery Kim (Division of Pediatric Hematology/Oncology Department of Pediatrics Asan Medical Center Children’s H) Eun Seok Choi (Division of Pediatric Hematology/Oncology Department of Pediatrics Asan Medical Center Children’s H) Kyung-Nam Koh (Division of Pediatric Hematology/Oncology Department of Pediatrics Asan Medical Center Children’s H) Ho Joon Im (Division of Pediatric Hematology/Oncology Department of Pediatrics Asan Medical Center Children’s H)

저널정보: 대한혈액학회 Blood Research Blood Research Vol.57 No.2

발행연도: 2022.6

수록면: 152 - 157 (6page)

DOI: 10.5045/br.2022.2022047

이용수

📌

연구주제

📖

연구배경

🔬

연구방법

🏆

연구결과

초록· 키워드

오류제보하기

Background The incorporation of a reduced-intensity conditioning (RIC) regimen in hematopoietic cell transplantation (HCT) for patients with hemophagocytic lymphohistiocytosis (HLH) has decreased early mortality but is associated with a high rate of mixed chimerism and graft failure. Here, we present a successful single-center experience using busulfan and a fludarabine-based RIC regimen for the treatment of HLH. Methods The medical records of pediatric patients with HLH who underwent HCT using a busulfan/ fludarabine-based RIC regimen between January 2008 and December 2017 were reviewed retrospectively. Results Nine patients received HCT with a busulfan/fludarabine-based RIC regimen. Three patients had primary HLH, and the other six patients had secondary HLH with multiple reactivations. All three patients with primary HLH had UNC13D mutations. All patients achieved neutrophil and platelet engraftment at a median of 11 days (range, 10?21) and 19 days (range, 13?32), and all eight evaluable patients had sustained complete donor chimerism at the last follow-up. Two patients (22%) experienced grade 2 acute graft-versus- host disease (GVHD). Two patients (22%) developed chronic GVHD, and one died from chronic GVHD. One patient (11%) experienced reactivation 4 months after HCT from a syngeneic donor and died of the disease. The 8-year overall survival and event-free survival rates were 78%. No early treatment-related mortality within 100 days after HCT was observed. Conclusion Our experience suggests that a busulfan/fludarabine-based RIC regimen is a viable option for pediatric patients with HLH who require HCT.

#Hematopoietic stem cell transplantation #Pediatric #Hemophagocytic lymphohistiocytosis

참고문헌 (0)

참고문헌 신청

참고문헌이 DBpia에서 서비스 중이라면, [참고문헌 신청]을 통해 등록해보세요

함께 읽어보면 좋을 논문

논문 유사도에 따라 DBpia 가 추천하는 논문입니다. 함께 보면 좋을 연관 논문을 확인해보세요!

이 논문의 저자 정보

Jin Kyung Suh

소속기관 Division of Pediatric Hematology/Oncology, Department of Pediatrics, Asan Medical Center Children’s

주요연구분야 의약학 > 임상의학 > 임상병리학

논문수 7 이용수 2

Young Kwon Koh