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家族性早发性血液恶性肿瘤聚集现象。

Familial aggregation of early-onset haematological malignancies.

机构信息

Finnish Cancer Registry, Institute for Statistical and Epidemiological Cancer Research, Helsinki, Finland.

Department of Internal Medicine, Helsinki University Hospital, Helsinki, Finland.

出版信息

Br J Haematol. 2021 Jun;193(6):1134-1141. doi: 10.1111/bjh.17477. Epub 2021 May 18.

Abstract

Population-based studies on familial aggregation of haematological malignancies (HM) have rarely focused specifically on early-onset HMs. We estimated standardized incidence ratios (SIR) and cumulative risks of relatives with Hodgkin lymphoma (HL), non-Hodgkin lymphomas (NHL), acute lymphoblastic leukaemia/lymphoma (ALL/LBL) and acute myeloid leukaemia (AML) when index persons and relatives were diagnosed with early-onset HM. A total of 8791 patients aged ≤40 years and diagnosed with primary HM in Finland from 1970 to 2012 were identified from the Finnish Cancer Registry and their 75 774 family members were retrieved from the population registry. SIRs for concordant HMs were elevated among first-degree relatives in all of the most common HMs of children and adolescents and young adults (AYA). The risk was highest among siblings with HL (SIR 9·09, 95% confidence interval 5·55-14·04) and AML (8·29, 1·00-29·96). HL also had the highest cumulative risk for siblings at ≤40 years of age (0·92% vs. 0·11% in the population). In conclusion, significantly elevated SIRs indicate a role of shared aetiological factors in some families, which should be noted in the clinical setting when caring for patients with early-onset HMs.

摘要

基于人群的血液恶性肿瘤(HM)家族聚集性研究很少专门针对早发性 HM。我们估计了指数病例及其亲属在诊断为早发性 HM 时患有霍奇金淋巴瘤(HL)、非霍奇金淋巴瘤(NHL)、急性淋巴细胞白血病/淋巴瘤(ALL/LBL)和急性髓系白血病(AML)的标准化发病比(SIR)和累积风险。从芬兰癌症登记处确定了 1970 年至 2012 年芬兰 8791 名年龄≤40 岁的原发性 HM 患者,并从人口登记处检索了他们的 75774 名亲属。在所有儿童和青少年以及年轻成人(AYA)中最常见的 HM 中,一级亲属中一致 HM 的 SIR 升高。HL 和 AML 同胞的风险最高(HL 的 SIR 为 9.09,95%置信区间为 5.55-14.04;AML 的 SIR 为 8.29,1.00-29.96)。HL 还导致≤40 岁同胞的累积风险最高(0.92%,人群中为 0.11%)。总之,显著升高的 SIR 表明一些家庭中存在共同的病因因素,在临床环境中应注意到这一点,以便照顾早发性 HM 患者。

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