Copyright 2008 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.2008
The study by Landgren et al1 investigated the risk of malignancy in patients with type 1 Gaucher disease (GD). We have several questions about their article that address the validity of the findings. First, we are concerned by the large number of patients with reported type 1 GD. The prevalence of type 1 GD is 1:40 000 to 60 000 in the general population and 1:500 to 800 among Ashkenazi Jews.2- 6 Recognizing that Ashkenazi Jews constitute no more than 2% to 3% of the US population, fewer than 200 patients with GD should be expected among 4.5 million Department of Veterans Affairs (VA) hospital patients. The authors found 1525. The authors attribute the high prevalence of GD to the study being hospital based. However, a national hospital-based study in Spain, using demographic, clinical, diagnostic, and radiological data for patient identification, found only 75 patients with GD7—numbers even lower than those predicted from other European studies.
Weinreb NJ, Andersson HC, Banikazemi M, Barranger J, Beutler E, Charrow J, Grabowski GA, Hollak CEM, Kaplan P, Mankin H, Mistry PK, Rosenbloom BE, vom Dahl S, Zimran A. Prevalence of Type 1 Gaucher Disease in the United States. Arch Intern Med. 2008;168(3):326-327. doi:10.1001/archinternmed.2007.128