Hairy cell leukaemia: a heterogeneous disease?
Hairy cell leukaemia: a heterogeneous disease?
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DOI：
10.1111/j.1365-2141.2008.07156.x
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发表时间：
2008-07-01
影响因子：
6.5
通讯作者：
Anderson, William F.
中科院分区：
文献类型：
--
作者：
Dores, Graca M.;Matsuno, Rayna K.;Anderson, William F.
美国国家癌症研究所的监测，流行病学和最终结果计划被用来开发毛细胞白血病（HCL）的病因学线索。描述性技术（年龄校正的发病率趋势，年龄特异性发病率（IR），诊断时的年龄分布）补充了数学模型（双组分混合，广义线性回归，年龄-阶段-队列）。1978-2004年共确诊HCL 2856例，发病率为0.32/10万人年。男性的IR比女性高近4倍，白人比黑人高3倍以上。随着时间的推移，时间趋势是稳定的。特定的IR迅速增加，直到大约40年，然后以较慢的速度上升。年龄特异性IR曲线反映了诊断时的双峰早发性和迟发性年龄分布（或密度图），性别之间存在一定差异。在男性和女性中，双组分混合模型比单一密度或癌症人群更好地拟合数据。在对时间趋势进行充分调整后，中期队列模型证实了统计学显著的年龄相关效应（中期和出生队列效应）。总之，年龄发病模式（率和双峰密度）表明HCL是一种异质性疾病，由至少两个潜在的亚组和/或癌症人群的发病年龄。不同的早发型和晚发型HCL人群可能反映了不同的年龄相关的因果途径，风险因素概况和/或干细胞来源。
The US National Cancer Institute's Surveillance, Epidemiology and End Results program was used to develop aetiological clues for hairy cell leukaemia (HCL). Descriptive techniques (age-adjusted incidence trends, age-specific incidence rates (IR), and age distributions-at-diagnosis) were supplemented with mathematical models (two-component mixture, generalized linear regression, and age-period-cohort). There were 2856 cases of HCL diagnosed during 1978-2004 (IR 0.32/100 000 person-years). IRs were nearly 4-fold greater among men than women and more than 3-fold higher for Whites than Blacks. Temporal trends were stable over time. Age-specific IRs increased rapidly until approximately 40 years then rose at a slower pace. The age-specific IR curves reflected bimodal early- and late-onset age distributions-at-diagnosis (or density plots), with some variation by gender. Among both men and women, a two-component mixture model fitted the data better than a single density or cancer population. Age-period-cohort models confirmed statistically significant age-related effects after full adjustment for temporal trends (calendar-period and birth-cohort effects). In summary, age incidence patterns (rates and bimodal densities) suggested that HCL is a heterogeneous disease, consisting of at least two underlying subgroups and/or cancer populations by age-at-onset. Distinct early- and late-onset HCL populations may reflect different age-related causal pathways, risk factor profiles, and/or stem cells of origin.