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Cancer in the National Cancer Institute inherited bone marrow failure syndrome cohort after fifteen years of follow-up

机译:经过15年的随访美国国家癌症研究所的癌症继承了骨髓衰竭综合征

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摘要

The National Cancer Institute Inherited Bone Marrow Failure Syndromes Cohort enrolls patients with the four major syndromes: Fanconi anemia, dyskeratosis congenita, Diamond-Blackfan anemia, and Shwachman-Diamond syndrome, and follows them with a common comprehensive protocol. The current analysis includes more than double the numbers of patients and person-years since our first report, published in 2010. Patients with Fanconi anemia and dyskeratosis congenita developed head and neck and anogenital squamous cell carcinomas at rates that were hundreds-fold greater than those of the general population. In competing risk analyses the cumulative incidence of severe bone marrow failure, leading to stem cell transplantation or death, was more than 70% by age 60. Patients with Diamond-Blackfan anemia developed lung, colon, and cervical cancer at rates greater than those of the general population. The cumulative incidence of severe bone marrow failure in those with Diamond-Blackfan anemia was 50% by age 60. The smaller group, with Shwachman-Diamond syndrome, have not as yet developed a significant number of solid tumors, but 40% developed bone marrow failure by age 50. The risk of solid tumors following stem cell transplantation in Fanconi anemia and in dyskeratosis congenita was significantly higher than in non-transplanted patients. There was no clear association of genotype with cancer in any of the syndromes. Cancer was most common in Fanconi anemia, followed by dyskeratosis congenita; Diamond-Blackfan anemia and Shwachman-Diamond syndrome are less cancer-prone, but nonetheless all patients are at increased risks of bone marrow failure and specific cancers. Identifier: 00027274
机译:美国国家癌症研究所遗传性骨髓衰竭综合征队列招募患有以下四种主要综合征的患者:Fanconi贫血,先天性角化不全,Diamond-Blackfan贫血和Shwachman-Diamond综合征,并按照通用的综合方案进行治疗。自我们于2010年发布第一份报告以来,当前的分析包括患者和人年数的两倍以上。患有范可尼贫血和先天性角化病的患者发生的头颈部和肛门生殖器鳞状细胞癌的发病率是其的数百倍。总人口。在竞争风险分析中,到60岁,导致干细胞移植或死亡的严重骨髓衰竭的累积发生率超过70%。Diamond-Blackfan贫血患者罹患肺癌,结肠癌和宫颈癌的比率大于普通人群。到60岁时,Diamond-Blackfan贫血患者中严重骨髓衰竭的累积发生率为50%。较小的Shwachman-Diamond综合征患者尚未发展出大量实体瘤,但40%的患者发展为骨髓到50岁时衰竭。在Fanconi贫血和先天性角化不全患者中,干细胞移植后发生实体瘤的风险明显高于未移植的患者。在任何综合征中,基因型与癌症均无明确关联。癌症最常见于范科尼贫血,其次为先天性角化不全。 Diamond-Blackfan贫血和Shwachman-Diamond综合征较不易患癌症,但所有患者的骨髓衰竭和特定癌症的风险均增加。识别码:00027274

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