Prevalence of familial malignancy in a prospectively screened cohort of patients with lymphoproliferative disorders.
Prevalence of familial malignancy in a prospectively screened cohort of patients with lymphoproliferative disorders.
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DOI：
10.1111/j.1365-2141.2008.07355.x
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发表时间：
2008-11
影响因子：
6.5
通讯作者：
Freedman AS
中科院分区：
文献类型：
--
作者：
Brown JR;Neuberg D;Phillips K;Reynolds H;Silverstein J;Clark JC;Ash M;Thompson C;Fisher DC;Jacobsen E;LaCasce AS;Freedman AS
越来越多的证据表明淋巴瘤的发生与遗传有关。本研究的目的是确定在三级护理中心就诊的连续淋巴瘤患者中家族性淋巴增生性恶性肿瘤的发生率，并招募有多个一级亲属受影响的家庭进行数据和组织收集研究。从2004年开始，Dana-Farber癌症研究所要求所有患有非霍奇金淋巴瘤或霍奇金淋巴瘤或慢性淋巴细胞白血病（CLL）的新患者完成一页经irb批准的自我管理的家族史问卷。在1948例可评估的患者中，55.4%报告有一级亲属患有恶性肿瘤，在CLL先证中比例最高。淋巴恶性肿瘤尤为常见，9.4%的先证者报告有一级亲属患有相关的LPD。CLL的这一频率再次最高，为13.3%的CLL先证者，而NHL先证者为8.8%，HL先证者为5.9% （p=0.002）。CLL先证者父母的CLL患病率显著增加（p < 0.05）， NHL先证者父亲患NHL的风险高于母亲（p=0.026）。我们的结论是，淋巴增生性疾病的家族性聚集在新诊断的患者中很常见，因诊断而有显着差异，并对人群疾病负担有意义。
Increasing evidence points to a heritable contribution to the development of lymphoma. The goal of this study was to determine the rate of familial lymphoproliferative malignancy among consecutive lymphoma patients presenting to a tertiary care center and to enroll families with multiple affected first degree relatives on a data and tissue collection study. Beginning in 2004 all new patients presenting to the Dana-Farber Cancer Institute with non-Hodgkin’s or Hodgkin’s lymphoma or chronic lymphocytic leukemia (CLL) were asked to complete a one-page IRB-approved self-administered family history questionnaire. 55.4% of 1948 evaluable patients reported a 1st degree relative with a malignancy, highest among CLL probands. Lymphoid malignancies were particularly common, with 9.4% of all probands reporting a 1st degree relative with a related LPD. This frequency was again highest for CLL, at 13.3% of CLL probands, compared to 8.8% of NHL probands and 5.9% of HL probands (p=0.002). The prevalence of CLL was significantly increased in parents of CLL probands (p < 0.05), and a greater risk of NHL was seen in fathers of NHL probands than in mothers (p=0.026). We conclude that familial aggregation of lymphoproliferative disorders is common among newly diagnosed patients, varies significantly by diagnosis and contributes meaningfully to the population disease burden.
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