Clinical characteristics of familial B-CLL in the National Cancer Institute Familial Registry
Clinical characteristics of familial B-CLL in the National Cancer Institute Familial Registry
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DOI：
10.3109/10428190109097681
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发表时间：
2001-06-01
影响因子：
2.6
通讯作者：
Caporso, NE
中科院分区：
文献类型：
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作者：
Ishibe, N;Sgambati, MT;Caporso, NE
在一项正在进行的研究中，通过医生和自我转介招募了一级亲属中有两个或更多活着的B-CLL病例的家庭。自1967年以来，国家癌症研究所(NCI)家族性B-CLL登记登记了28个家系，73例B-CLL。医疗、临床和人口统计信息已从私人医生、患者访谈、医院记录和死亡证明中获得。我们使用SEER注册数据来比较散发性B-CLL和家族性B-CLL的特征。家族性病例的平均确诊年龄(57.9+/-12.1)比散发性病例(70.1+/-11.9)年轻约10岁。家族性慢性淋巴细胞性白血病病例中第二原发谣言的比例也高于散发性病例(16%比8.8%)。然而，向非霍奇金淋巴瘤的转化率似乎与报告的散发性病例没有什么不同。总而言之，我们观察到家族性和散发性病例之间的一些差异；这些特征是否会影响生存时间或疾病的严重程度尚不清楚。对多发性B-CLL家系的研究将有助于描述可能在两种形式的B-CLL的发展中发挥作用的基因和环境因素。
In an ongoing study, families with two or more living cases of B-CLL in first-degree relatives have been recruited through physician and self-referral. Since 1967, 28 kindreds with 73 cases of B-CLL have been enrolled within the National Cancer Institute (NCI) Familial B-CLL Registry. Medical, clinical, and demographic information have been obtained from private physicians, patient interview, hospital records, and death certificates. We used SEER Registry data to compare characteristics of sporadic B-CLL to familial B-CLL. The mean age at diagnosis was approximately 10 years younger among familial cases (57.9 +/- 12.1) than that observed in sporadic cases (70.1 +/- 11.9). A higher percentage of second primary rumors among familial CLL cases compared to reports in sporadic was also observed (16% vs. 8.8%). However, the transformation rate to non-Hodgkin's lymphoma does not appear to be different from that reported for sporadic cases. In conclusion, we observed some differences between familial and sporadic cases; whether any of these characteristics affect survival time or severity of disease is unknown. The study of families with multiple B-CLL cases will aid in delineating the genes and environmental factors that may play a role in the development of both forms of B-CLL.