Author(s): Shiran A, Brenner B, Laor A, Tatarsky I
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Abstract BACKGROUND: An increased incidence of cancer, especially hematopoietic in origin, has long been suspected but never established in patients with Gaucher disease. METHODS: To determine whether patients with Gaucher disease have an increased risk of cancer, the authors conducted a retrospective cohort study, comparing the incidence and type of cancer in 48 patients with Gaucher disease with those of 511 control subjects without the disease. RESULTS: Among patients with Gaucher disease, 10 of 48 (20.8\%) had cancer, as compared with 35 of 511 (6.8\%) of the control group (P = 0.0027; relative risk, 3.6; 95\% confidence interval, 1.7-7.5). As compared with the control group, patients with Gaucher disease had a 14.7-fold risk of having cancer of hematopoietic origin (10.4\% [5 of 48] versus 0.78\% [4 of 511], respectively; P = 0.00037; 95\% confidence interval, 5.2-41.7). The mean age at cancer diagnosis in the group with Gaucher disease was 57 +/- 18 years. CONCLUSIONS: The authors conclude that patients with Gaucher disease have a significantly increased risk of cancer, occurring in late adulthood. Of all the cancers, hematologic cancers are significantly more prevalent.
This article was published in Cancer
and referenced in Journal of Biometrics & Biostatistics