Increased risk of cancer in patients with Gaucher disease.
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.
Shiran
A
A
Institute of Hematology, Rambam Medical Center, Haifa, Israel.
Brenner
B
B
Laor
A
A
Tatarsky
I
I
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015331:Cohort Studies; D005260:Female; D005776:Gaucher Disease / Q000150:complications* / Q000401:mortality / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000150:complications* / Q000401:mortality / Q000473:pathology; D012189:Retrospective Studies; D013163:Splenomegaly / Q000209:etiology; D016019:Survival Analysis