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.
journal_name
Cancerjournal_title
Cancerauthors
Shiran A,Brenner B,Laor A,Tatarsky Idoi
10.1002/1097-0142(19930701)72:1<219::aid-cncr28207subject
Has Abstractpub_date
1993-07-01 00:00:00pages
219-24issue
1eissn
0008-543Xissn
1097-0142journal_volume
72pub_type
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