Abstract

HCL has been considered to be a disease of predominantly young males with, after the introduction of effective treatment, excellent prognosis. However, although very effective, these therapies introduced in the 1980’s are not curative. The Swedish Lymphoma Registry records all patients diagnosed since year 2000, with a 96 % coverage compared to the Swedish Cancer Registry, which is compulsory since 60 years. In contrast, previously published studies include patients highly selected for inclusion in treatment studies or referral to major medical centers. The Swedish registry data confirms the high male to female ratio, with yearly incidences of 5.3 males and 1.1 females per million. However, the median age at diagnosis was 62 years (range 30–92). The incidence in males rise with age as follows: 3 (30–49 yrs), 11 (50–69 yrs), 16 (70–79 yrs), and 20/million (80–95yrs). The 5-year overall survival of these unselected patients was 81%. Age and performance status had strong influence on survival. If HCL was diagnosed before age 60 the 5-year survival was 94%, whereas it was 70% for patients over 60 years. We also performed a 15-year follow-up of patients included in our first cladribine trials in the early 1990’s. The progression-free survival at 10 years was 60% and at 15 years 55%, irrespective of age. However, the 10 year and 15 year overall survivals were 91% and 82% for patients <60 yrs, in contrast to 65% and 42% for those with diagnosis after age 60 years. We conclude that there is a proportion of older patients with HCL with a similar progressionfree survival after cladribine treatment as the younger, but poorer overall survival. Further analyses, including epidemiology of unselected total populations diagnosed since 1987, age-adjusted survival estimates, and incidence of secondary cancers among the HCL patients will be presented.

Disclosures: No relevant conflicts of interest to declare.

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