Kendrick Tulene S, Buic Daria, Fuller Kathy A, Erber Wendy N
Haematology Department, Royal Perth Hospital, Perth, Australia.
School of Biomedical Sciences, The University of Western Australia, Crawley, Australia.
Ann Lab Med. 2025 Mar 1;45(2):133-145. doi: 10.3343/alm.2024.0477. Epub 2025 Jan 8.
Chromosomes 5 and 7 are large chromosomes that contain close to 1,000 genes each. Deletions of the long arms or loss of the entire chromosome (monosomy) are common defects in myeloid disorders, particularly MDS and AML. Loss of material from either chromosome 5 or 7 results in haploinsufficiency of multiple genes, with some implicated in leukemogenesis. Abnormalities of one or both occur in up to 15% of MDS and AML cases and co-segregate in half of these. Generally, these chromosomal abnormalities are harbingers of adverse risk in both myeloid disorders. A notable exception is del(5q) in 5q- syndrome, a subtype of MDS. In this review, we describe the pathogenesis and genetic consequences of deletions in chromosomes 5 and 7. Furthermore, we provide an overview of current testing methodologies used in the assessment of these chromosomal defects in hematological malignancies and describe the disease associations and prognostic implications of aberrations in chromosomes 5 and 7 in both MDS and AML.
5号和7号染色体是大型染色体,每条染色体包含近1000个基因。长臂缺失或整条染色体丢失(单体性)是髓系疾病,尤其是骨髓增生异常综合征(MDS)和急性髓系白血病(AML)常见的缺陷。5号或7号染色体物质丢失会导致多个基因单倍体不足,其中一些基因与白血病发生有关。在高达15%的MDS和AML病例中会出现一条或两条染色体异常,且其中一半会共同分离。一般来说,这些染色体异常是两种髓系疾病不良风险的预兆。一个显著的例外是MDS的一个亚型——5q-综合征中的del(5q)。在本综述中,我们描述了5号和7号染色体缺失的发病机制和遗传后果。此外,我们概述了目前用于评估血液系统恶性肿瘤中这些染色体缺陷的检测方法,并描述了5号和7号染色体畸变在MDS和AML中的疾病关联及预后意义。