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Hairy cell leukaemia: a heterogeneous disease?

Abstract：

: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.

journal_name

Br J Haematol

journal_title

British journal of haematology

authors

Dores GM,Matsuno RK,Weisenburger DD,Rosenberg PS,Anderson WF

doi

10.1111/j.1365-2141.2008.07156.x

subject

Has Abstract

pub_date

2008-07-01 00:00:00

pages

45-51

issue

1

eissn

0007-1048

issn

1365-2141

pii

BJH7156

journal_volume

142

pub_type

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