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小儿骨髓增生异常综合征中的7号染色体单体

Monosomy 7 in Pediatric Myelodysplastic Syndromes.

作者信息

Wlodarski Marcin W, Sahoo Sushree S, Niemeyer Charlotte M

机构信息

Department of Pediatrics and Adolescent Medicine, Division of Pediatric Hematology and Oncology, Faculty of Medicine, Medical Center, University of Freiburg, Mathildenstr.1, Freiburg 79106, Germany; German Cancer Consortium (DKTK), Freiburg, Germany; Department of Hematology, St. Jude Children's Research Hospital, Memphis, TN, USA.

Department of Pediatrics and Adolescent Medicine, Division of Pediatric Hematology and Oncology, Faculty of Medicine, Medical Center, University of Freiburg, Mathildenstr.1, Freiburg 79106, Germany; Faculty of Biology, University of Freiburg, Freiburg, Germany; Spemann Graduate School of Biology and Medicine, University of Freiburg, Albertstr.19A, Freiburg 79104, Germany.

出版信息

Hematol Oncol Clin North Am. 2018 Aug;32(4):729-743. doi: 10.1016/j.hoc.2018.04.007.

Abstract

Myelodysplastic syndromes (MDS) in children and adolescents are a rare heterogeneous group of clonal stem cell disorders. Complete or partial loss of chromosome 7 constitutes the most common cytogenetic abnormality encountered in any type of childhood MDS, is associated with more advanced disease, and usually requires a timely allogeneic stem cell transplantation. This article provides insights into the current understanding of the genotype, phenotype, and clonal evolution patterns in pediatric MDS associated with loss of chromosome 7.

摘要

儿童和青少年的骨髓增生异常综合征(MDS)是一组罕见的异质性克隆性干细胞疾病。7号染色体的完全或部分缺失是任何类型儿童MDS中最常见的细胞遗传学异常,与更晚期的疾病相关,通常需要及时进行异基因干细胞移植。本文深入探讨了目前对与7号染色体缺失相关的儿童MDS的基因型、表型和克隆进化模式的理解。

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