Anderson Lesley A, Pfeiffer Ruth M, Rapkin Joshua S, Gridley Gloria, Mellemkjaer Lene, Hemminki Kari, Björkholm Magnus, Caporaso Neil E, Landgren Ola
Viral Epidemiology Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, NIH, Bethesda, MD 20892, USA.
J Clin Oncol. 2008 Oct 20;26(30):4958-65. doi: 10.1200/JCO.2007.14.6571. Epub 2008 Jul 7.
Genetic factors are important in the etiology and pathogenesis of chronic lymphocytic leukemia (CLL), Hodgkin's lymphoma (HL), and non-Hodgkin's lymphoma (NHL). Only a few small studies have assessed clinical characteristics and prognosis for familial patients, with inconsistent findings.
Using population-based registries from Sweden and Denmark, 7,749 patients with CLL, 7,476 patients with HL, and 25,801 patients with NHL with linkable first-degree relatives were identified. Kaplan-Meier curves were constructed to compare survival in patients with lymphoma with and without a family history of lymphoma. The risk of dying was assessed using adjusted Cox proportional hazard models.
We found 85 patients with CLL (1.10%), 95 patients with HL (1.28%), and 206 patients with NHL (0.80%) with a family history of any lymphoma. Five-year mortality was similar for patients with CLL (hazard ratio [HR], 1.28; 95% CI, 0.95 to 1.72), HL (HR, 0.78; 95% CI, 0.49 to 1.25), and NHL (HR, 0.91; 95% CI, 0.74 to 1.12) versus without a family history of any lymphoma. Mortality was also similar for patients with versus without a family history of the same lymphoma. T-cell/anaplastic lymphoma patients with a family history of NHL had poorer outcome 5-years after diagnosis (HR, 5.38; 95% CI, 1.65 to 17.52). Results were similar for 10 years of follow-up.
With the exception of T-cell/anaplastic lymphoma, survival patterns for patients with CLL, HL, and NHL with a family history of lymphoma were similar to those for sporadic patients, suggesting that most familial lymphomas do not have an altered clinical course. Our findings provide no evidence to modify therapeutic strategies for patients with CLL, HL, or NHL based solely on family history.
遗传因素在慢性淋巴细胞白血病(CLL)、霍奇金淋巴瘤(HL)和非霍奇金淋巴瘤(NHL)的病因学和发病机制中起着重要作用。仅有少数小型研究评估了家族性患者的临床特征和预后,结果并不一致。
利用瑞典和丹麦基于人群的登记系统,确定了7749例CLL患者、7476例HL患者和25801例NHL患者,这些患者均有可关联的一级亲属。构建Kaplan-Meier曲线以比较有和没有淋巴瘤家族史的淋巴瘤患者的生存率。使用校正后的Cox比例风险模型评估死亡风险。
我们发现85例CLL患者(1.10%)、95例HL患者(1.28%)和206例NHL患者(0.80%)有任何淋巴瘤家族史。有CLL(风险比[HR],1.28;95%置信区间,0.95至1.72)、HL(HR,0.78;95%置信区间,0.49至1.25)和NHL(HR,0.91;95%置信区间,0.74至1.12)家族史的患者与无任何淋巴瘤家族史的患者相比,五年死亡率相似。有和没有相同淋巴瘤家族史的患者死亡率也相似。有NHL家族史的T细胞/间变性淋巴瘤患者在诊断后5年预后较差(HR, 5.38;95%置信区间,1.65至17.52)。10年随访结果相似。
除T细胞/间变性淋巴瘤外,有淋巴瘤家族史的CLL、HL和NHL患者的生存模式与散发性患者相似,这表明大多数家族性淋巴瘤的临床病程没有改变。我们的研究结果没有提供仅基于家族史而改变CLL、HL或NHL患者治疗策略的证据。