A population-based study of hairy cell leukemia in Israel

Ora Paltiel*, Bella Adler, Micha Barchana, Eldad J. Dann

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

31 Scopus citations

Abstract

Objectives: Few population-based data exist on the incidence and prognosis of hairy cell leukemia (HCL). Our objectives were to study the effect of socio-demographic factors on this rare disease and the risk of second malignancies occurring in HCL patients. Methods: We measured crude and age-adjusted incidence rates of HCL based on reporting to the Israel Cancer Registry (ICR) 1991-2001. Using Kaplan-Meier and multivariate analysis, we assessed survival by gender, ethnicity and geographic region. We ascertained additional primary tumors reported in this population and calculated standardized incidence ratios (SIRs) for tumors reported after the diagnosis of HCL. Results: The ICR registered 147 cases of HCL among males and 34 in females between 1991 and 2001. Age-adjusted incidence rates were 1.62/10 6/yr for women and 7.97/10 6/yr for men, with rates 1.5 times higher in Jewish than in non-Jewish (mainly Arab) men. Mean overall survival also differed by ethnicity. In a multivariate model, increasing age at diagnosis (P < 0.001), as well as Arab origin (P = 0.008) were associated with poorer survival but gender did not significantly affect the survival after controlling for age and ethnicity. Other primary malignancies were reported in 20 (11%) individuals, with a predominance of genito-urinary tumors (65%) among males. Secondary genito-urinary tumors were significantly increased above the expected population rates (SIR 3.23, 95% confidence interval: 1.39-6.36, P = 0.008). Conclusions: In the Israeli population, age and ethnicity were associated with prognosis of HCL. Variations in disease characteristics, stage of disease at diagnosis or differential access to treatment may contribute to these findings. Patients with HCL appear to be at increased risk for genito-urinary malignancies.

Original languageAmerican English
Pages (from-to)372-377
Number of pages6
JournalEuropean Journal of Haematology
Volume77
Issue number5
DOIs
StatePublished - Nov 2006
Externally publishedYes

Keywords

  • Cancer registry
  • Epidemiology
  • Hairy cell leukemia
  • Second malignancy

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