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范可尼样贫血与 FANCM 突变相关。

Fanconi-like anemia related to a FANCM mutation.

机构信息

Servicio de Oncología Radioterápica Hospital Clínico Universitario Virgen de la Arrixaca, Murcia, Spain.

Servicio de Oncología Médica Hospital Clínico Universitario Virgen de la Arrixaca, Murcia, Spain.

出版信息

Eur J Med Genet. 2022 Jan;65(1):104399. doi: 10.1016/j.ejmg.2021.104399. Epub 2021 Nov 15.

DOI:10.1016/j.ejmg.2021.104399
PMID:34793962
Abstract

Fanconi anemia is primarily inherited as an autosomal recessive genetic disorder with common delays in diagnosis and challenging treatments. Fanconi anemia patients have a high risk of developing solid tumors, particularly in the head and neck or anogenital regions. The diagnosis of Fanconi anemia is primarily based on the chromosomal breakage but FA gene sequencing is recommended in all patients with a positive chromosome fragility test. Here, we present a 32-year-old man with advanced tonsil squamous cell carcinoma and fatal toxicity after the first cycle of chemotherapy. No anemia was present. A recent variant mutation if the FANCM gene was detected (c1511_1515delGAGTA (pArg504AsnfsTer29)). Homozygous or double heterozygous pathogenic variants have been reported in FANCM and linked to azoospermia and primary ovarian failure without anemia. Alterations in this gene have also been associated with a genetic predisposition for solid tumors (breast and ovarian cancer) and hematological malignancies (B-cell acute lymphoblastic leukemia). Due to the hypersensitivity of these patients to DNA-damaging agents such as chemotherapy and radiotherapy, surgery is the best treatment option for malignant solid tumors. Dose reductions or alternative regimens of chemotherapy and/or radiotherapy are recommended in FA patients who develop a malignant tumor.

摘要

范可尼贫血症主要作为一种常染色体隐性遗传疾病遗传,其诊断通常会延迟,治疗也极具挑战性。范可尼贫血症患者有很高的罹患实体肿瘤的风险,尤其是在头颈部或肛生殖区域。范可尼贫血症的诊断主要基于染色体断裂,但建议所有染色体脆性试验阳性的患者进行 FA 基因测序。在此,我们报告了一例 32 岁男性,患有晚期扁桃体鳞状细胞癌,在第一周期化疗后出现致命毒性。没有贫血。最近检测到 FANCM 基因的变异突变(c1511_1515delGAGTA(pArg504AsnfsTer29))。FANCM 中的纯合子或双杂合子致病性变异已被报道,并与无贫血的无精子症和原发性卵巢功能衰竭相关。该基因的改变也与实体肿瘤(乳腺癌和卵巢癌)和血液系统恶性肿瘤(B 细胞急性淋巴细胞白血病)的遗传易感性相关。由于这些患者对化疗和放疗等 DNA 损伤剂高度敏感,因此手术是治疗恶性实体肿瘤的最佳选择。对于发生恶性肿瘤的 FA 患者,建议减少化疗和/或放疗的剂量或选择替代方案。

相似文献

1
Fanconi-like anemia related to a FANCM mutation.范可尼样贫血与 FANCM 突变相关。
Eur J Med Genet. 2022 Jan;65(1):104399. doi: 10.1016/j.ejmg.2021.104399. Epub 2021 Nov 15.
2
Individuals with FANCM biallelic mutations do not develop Fanconi anemia, but show risk for breast cancer, chemotherapy toxicity and may display chromosome fragility.具有 FANCM 双等位基因突变的个体不会患上范可尼贫血,但会增加乳腺癌、化疗毒性的风险,并且可能表现出染色体脆弱性。
Genet Med. 2018 Apr;20(4):452-457. doi: 10.1038/gim.2017.123. Epub 2017 Aug 24.
3
Biallelic truncating FANCM mutations cause early-onset cancer but not Fanconi anemia.双等位基因截断 FANCM 突变导致早发性癌症,但不会导致范可尼贫血。
Genet Med. 2018 Apr;20(4):458-463. doi: 10.1038/gim.2017.124. Epub 2017 Aug 24.
4
Assessing the spectrum of germline variation in Fanconi anemia genes among patients with head and neck carcinoma before age 50.评估50岁之前的头颈癌患者中范可尼贫血基因的种系变异谱。
Cancer. 2017 Oct 15;123(20):3943-3954. doi: 10.1002/cncr.30802. Epub 2017 Jul 5.
5
Squamous cell carcinomas of the head and neck in Fanconi anemia: risk, prevention, therapy, and the need for guidelines.范可尼贫血患者头颈部鳞状细胞癌:风险、预防、治疗及制定指南的必要性
Klin Padiatr. 2012 Apr;224(3):132-8. doi: 10.1055/s-0032-1308989. Epub 2012 Apr 13.
6
Mutational analysis of FANCL, FANCM and the recently identified FANCI suggests that among the 13 known Fanconi Anemia genes, only FANCD1/BRCA2 plays a major role in high-risk breast cancer predisposition.对FANCL、FANCM以及最近发现的FANCI进行的突变分析表明,在13个已知的范可尼贫血基因中,只有FANCD1/BRCA2在高危乳腺癌易感性中起主要作用。
Carcinogenesis. 2009 Nov;30(11):1898-902. doi: 10.1093/carcin/bgp218. Epub 2009 Sep 8.
7
Fanconi's anemia in adulthood: chemoradiation-induced bone marrow failure and a novel FANCA mutation identified by targeted deep sequencing.成人范可尼贫血:放化疗诱发的骨髓衰竭及通过靶向深度测序鉴定出的一种新型FANCA突变
J Clin Oncol. 2011 Jul 10;29(20):e591-4. doi: 10.1200/JCO.2011.35.1064. Epub 2011 Apr 25.
8
Defects in the Fanconi Anemia Pathway and Chromatid Cohesion in Head and Neck Cancer.范可尼贫血途径缺陷与头颈部癌症的染色单体黏合
Cancer Res. 2015 Sep 1;75(17):3543-53. doi: 10.1158/0008-5472.CAN-15-0528. Epub 2015 Jun 29.
9
Fanconi anemia: a model disease for studies on human genetics and advanced therapeutics.范可尼贫血:用于人类遗传学和先进治疗学研究的模型疾病。
Curr Opin Genet Dev. 2015 Aug;33:32-40. doi: 10.1016/j.gde.2015.07.002. Epub 2015 Aug 6.
10
Impaired FANCD2 monoubiquitination and hypersensitivity to camptothecin uniquely characterize Fanconi anemia complementation group M.FANCD2单泛素化受损以及对喜树碱超敏是范可尼贫血互补组M的独特特征。
Blood. 2009 Jul 2;114(1):174-80. doi: 10.1182/blood-2009-02-207811. Epub 2009 May 7.

引用本文的文献

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Homozygous FANCM Variant c.5101C>T p.(Gln1701*) in a Patient With Early Onset Breast Cancer, Chemotherapy Toxicity, and Chromosome Fragility: A Case Report.一名早发性乳腺癌、化疗毒性和染色体脆性患者的纯合子FANCM变异c.5101C>T p.(Gln1701*):病例报告
Cancer Rep (Hoboken). 2025 Aug;8(8):e70283. doi: 10.1002/cnr2.70283.
2
FANCM Gene Variants in a Male Diagnosed with Sertoli Cell-Only Syndrome and Diffuse Astrocytoma.FANCM 基因突变致男性单纯型支持细胞综合征伴弥漫性星形细胞瘤 1 例
Genes (Basel). 2024 May 28;15(6):707. doi: 10.3390/genes15060707.
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has dual roles in the limiting of meiotic crossovers and germ cell maintenance in mammals.
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Cell Genom. 2023 Jun 29;3(8):100349. doi: 10.1016/j.xgen.2023.100349. eCollection 2023 Aug 9.