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Increased risk of cancer in patients with gaucher disease
Author(s) -
Shiran Avinoam,
Brenner Benjamin,
Laor Arie,
Tatarsky Ilana
Publication year - 1993
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19930701)72:1<219::aid-cncr2820720139>3.0.co;2-y
Subject(s) - medicine , disease , cancer , confidence interval , incidence (geometry) , relative risk , retrospective cohort study , cohort , gastroenterology , physics , optics
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. Cancer 1993; 72:219–24.