University of Helsinki, Helsinki, Finland.
J Clin Oncol. 2013 Mar 1;31(7):938-43. doi: 10.1200/JCO.2012.43.5958. Epub 2013 Jan 2.
Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is one of the two established Hodgkin lymphoma (HL) subtypes. The risk factors of NLPHL are largely unknown. In general, genetic factors are known to have a modest effect on the risk of HL; however, familial risk in NLPHL has not been previously examined. We conducted a population-based study by using the Finnish registries and evaluated the familial risk in NLPHL.
We launched a population-based search to identify patients with NLPHL and their relatives by examining the records of the Finnish Cancer Registry, established in 1953, and the official Finnish population registries. We collected a data set of 692 patients with NLPHL, identified their 4,280 first-degree relatives, and calculated the registry-based standardized incidence ratios (SIRs) for different cancers in the first-degree relatives. In addition, the primary tumor biopsies of HL-affected relatives were collected when possible, the HL diagnoses were re-reviewed by a hematopathologist, and the SIR for NLPHL was calculated on the basis of confirmed NLPHL diagnoses.
On the basis of confirmed NLPHL diagnoses, the SIR for NLPHL was 19 (95% CI, 8.8 to 36) in the first-degree relatives. The risk was most prominent in female relatives of young patients. The registry-based SIR for classical HL was 5.3 (95% CI, 3.0 to 8.8), and for non-Hodgkin lymphoma, it was 1.9 (95% CI, 1.3 to 2.6).
Our results implicate an unexpectedly high familial component in the development of NLPHL. Research is warranted to identify the putative genetic and environmental factors underlying this finding and to develop strategies for better management of patients with NLPHL and their relatives.
结节性淋巴细胞为主型霍奇金淋巴瘤（NLPHL）是两种已确立的霍奇金淋巴瘤（HL）亚型之一。NLPHL 的危险因素在很大程度上尚不清楚。一般来说，遗传因素被认为对 HL 的风险有适度的影响；然而，NLPHL 的家族风险尚未被研究过。我们通过使用芬兰的登记处进行了一项基于人群的研究，并评估了 NLPHL 的家族风险。
我们通过检查成立于 1953 年的芬兰癌症登记处和官方芬兰人口登记处的记录，发起了一项基于人群的搜索，以确定 NLPHL 患者及其亲属。我们收集了一组 692 例 NLPHL 患者的数据，确定了他们的 4280 名一级亲属，并计算了一级亲属中不同癌症的基于登记处的标准化发病比（SIR）。此外，当可能时收集了受 HL 影响的亲属的原发性肿瘤活检，由血液病理学家重新审查 HL 诊断，并根据确诊的 NLPHL 诊断计算 NLPHL 的 SIR。
根据确诊的 NLPHL 诊断，一级亲属中 NLPHL 的 SIR 为 19（95%CI，8.8 至 36）。风险在年轻患者的女性亲属中最为突出。基于登记处的经典 HL 的 SIR 为 5.3（95%CI，3.0 至 8.8），而非霍奇金淋巴瘤的 SIR 为 1.9（95%CI，1.3 至 2.6）。
我们的结果表明，NLPHL 的发生存在意外高的家族成分。有必要进行研究，以确定这一发现背后的潜在遗传和环境因素，并制定更好地管理 NLPHL 患者及其亲属的策略。
