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[家族性白血病的诊断、监测与管理]

[Diagnosis, surveillance, and management of familial leukemia].

作者信息

Moritake Hiroshi

机构信息

Division of Pediatrics, Department of Developmental and Urological-Reproductive Medicine, Faculty of Medicine, University of Miyazaki.

出版信息

Rinsho Ketsueki. 2018;59(10):2290-2299. doi: 10.11406/rinketsu.59.2290.

Abstract

Recently, the modern technique of comprehensive genomic analysis has identified both somatic mutations originating from tumor cells and germline mutations as causative genes of inherited familial leukemias among which Fanconi anemia and Li-Fraumeni syndrome are well known. Pathogenic germline mutations occur in various pathways, affecting DNA repair, ribosome biogenesis, telomere biology, hematopoietic transcription factors, tumor suppressors, neutrophil development, and other critical cellular processes. The clinical manifestations of germline mutations present a wide phenotypic spectrum of patients displaying congenital anomalies, early-onset myelodysplastic syndrome, or no medical problems until the developing leukemia. The use of genetic tests to identify these affected persons will significantly benefit cancer surveillance and subsequent therapeutic interventions. Although familial leukemia treatment usually focuses on children, it is important for clinicians to recognize that familial leukemias can occur at any age, even among older adults. Genetic counseling after diagnosis is essential, and an immediate referral to experts in each disease is recommended.

摘要

最近,综合基因组分析的现代技术已确定源自肿瘤细胞的体细胞突变和种系突变都是遗传性家族性白血病的致病基因,其中范可尼贫血和李-佛美尼综合征最为人所知。致病性种系突变发生在各种途径中,影响DNA修复、核糖体生物合成、端粒生物学、造血转录因子、肿瘤抑制因子、中性粒细胞发育以及其他关键的细胞过程。种系突变的临床表现呈现出广泛的表型谱,患者表现为先天性异常、早发性骨髓增生异常综合征,或者在患白血病之前没有任何医学问题。使用基因检测来识别这些受影响的人将显著有益于癌症监测和后续的治疗干预。尽管家族性白血病的治疗通常侧重于儿童,但临床医生必须认识到家族性白血病可发生于任何年龄,甚至是老年人。诊断后的遗传咨询至关重要,建议立即转诊至每种疾病的专家处。

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