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Ola Landgren, MD, PhD; Ingemar Turesson, MD, PhD; Gloria Gridley, MS; Neil E. Caporaso, MD

Arch Intern Med. 2007;167(11):1189-1194.

Background  Some, but not all, reports suggest that patients with Gaucher disease are at increased risk of developing malignancies, particularly hematopoietic tumors. The aim of this study was to assess the pattern of Gaucher disease and subsequent malignancies among male veterans admitted to US Veterans Affairs hospitals.

Methods  Among 832 294 African American and 3 668 983 white male veterans with at least 1 hospital admission in US Veterans Affairs hospitals and up to 27 years of follow-up, we identified a total of 1525 patients with Gaucher disease; 11.7% were African Americans. We used Poisson regression methods for cohort data to estimate relative risks (RRs) and 95% confidence intervals (CIs) after adjusting for attained age and calendar year, race, number of hospital visits, and latency.

Results  When patients with Gaucher disease were compared with patients without Gaucher disease, the RR of any cancer was 0.91 (95% CI, 0.76-1.08 [n = 137]). When we stratified our analyses by race, risks were similar for whites (RR, 0.89; 95% CI, 0.74-1.07 [n = 120]) and African Americans (RR, 1.00; 95% CI, 0.61-1.64 [ n = 17]). Patients with Gaucher disease had an elevated risk for non-Hodgkin lymphoma (RR, 2.54; 95% CI, 1.32-4.88 [n = 9]), malignant melanoma (RR, 3.07; 95% CI, 1.28-7.38 [n = 5]), and pancreatic cancer (RR, 2.37; 95% CI, 1.13-4.98 [n = 7]). Among the remaining 19 cases involving defined solid tumors and 7 other hematologic malignancies, we found no statistical association with Gaucher disease.

Conclusion  We found 2- to 3-fold risks of non-Hodgkin lymphoma, malignant melanoma, and pancreatic cancer in patients with Gaucher disease, but no significant association between Gaucher disease and cancer in general or with other specific malignancies such as multiple myeloma.

Author Affiliations: Division of Cancer Epidemiology and Genetics, National Cancer Institute, Bethesda, Md (Drs Landgren, Gridley, and Caporaso); and Section of Hematology, Department of Medicine, Malmö University Hospital, University of Lund, Malmö, Sweden (Dr Turesson).

RELATED LETTERS

Prevalence of Type 1 Gaucher Disease in the United States
Neal J. Weinreb, Hans C. Andersson, Maryam Banikazemi, John Barranger, Ernest Beutler, Joel Charrow, Gregory A. Grabowski, Carla E. M. Hollak, Paige Kaplan, Henry Mankin, Pramod K. Mistry, Barry E. Rosenbloom, Stephan vom Dahl, and Ari Zimran
Arch Intern Med. 2008;168(3):326-327.
EXTRACT | FULL TEXT  

Prevalence of Type 1 Gaucher Disease in the United States—Reply
Ola Landgren, Gloria Gridley, Neil E. Caporaso, and Ingemar Turesson
Arch Intern Med. 2008;168(3):327-328.
EXTRACT | FULL TEXT  

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Prevalence of Type 1 Gaucher Disease in the United States
Weinreb et al.
Arch Intern Med 2008;168:326-327.
FULL TEXT  

Prevalence of Type 1 Gaucher Disease in the United States--Reply
Landgren et al.
Arch Intern Med 2008;168:327-328.
FULL TEXT