异基因造血干细胞移植联合骨髓间充质干细胞输注治疗Shwachman-Diamond综合征合并骨髓增生异常综合征1例并文献复习

Allogeneic hematopoietic stem cell transplantation with bone marrow derived mesenchymal stem cell co-infusion in Shwachman-Diamond syndrome with myelodysplastic syndrome:A case report and literature review

郑晓丽 ¹丁丽 ¹闫洪敏 ¹韩冬梅 ¹李晟 ¹刘静 ¹王恒湘¹

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作者信息

1. 中国人民解放军空军特色医学中心血液病科(北京,100142)
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摘要

Shwachman-Diamond综合征(Shwachman-Diamond syndrome,SDS)是一种临床罕见的多系统累及的疾病,主要表现为骨髓衰竭、胰腺外分泌功能不全及骨骼发育异常,骨髓衰竭可为再生障碍性贫血、骨髓增生异常综合征,部分可进展为急性白血病,预后欠佳.文章报道1例外周血造血干细胞移植联合骨髓间充质干细胞输注治疗SDS合并骨髓增生异常综合征患者的诊疗经过.造血干细胞移植是治疗SDS并发造血系统疾病的有效手段,对于有造血系统并发症的患者应尽早进行造血干细胞移植,可改善疾病预后.

Abstract

Shwachman-Diamond syndrome(SDS)is a rare,multi-systemic disorder with bone marrow failure,exocrine pancreatic insufficiency,and skeletal abnormalities.Bone marrow failure include severe aplastic anemia and myelodysplastic syndrome,some patients develop acute leukemia with poor prognosis.It reported a case of SDS with myelodysplastic syndrome successfully treated with allogeneic hematopoietic stem cell transplantation and bone marrow derived mesenchymal stem cell co-infusion.Allogeneic hematopoietic stem cell transplantation is a feasible and effective option for SDS patients with bone marrow failure.Early recognition of the disease and hematopoietic stem cell transplantation for SDS patients with bone marrow failure will improve survival.

关键词

造血干细胞移植/间充质干细胞/Shwachman-Diamond综合征

Key words

hematopoietic stem cell transplantation/mesenchymal stem cell/Shwachman-Diamond syndrome

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出版年

2024

临床血液学杂志

华中科技大学同济医学院血液病研究所北京医科大学血液病研究所

临床血液学杂志

CSTPCD

影响因子：1.063

ISSN：1004-2806

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