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- Landgren, Ola, et al.
(author)
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Risk of malignant disease among 1525 adult male US veterans with gaucher disease
- 2007
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In: Archives of Internal Medicine. - 0003-9926. ; 167:11, s. 1189-1194
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Journal article (peer-reviewed)abstract
- 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.
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- Landgren, Ola, et al.
(author)
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Risk of plasma cell and lymphoproliferative disorders among 14621 first-degree relatives of 4458 patients with monoclonal gammopathy of undetermined significance in Sweden.
- 2009
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In: Blood. - : American Society of Hematology. - 0006-4971 .- 1528-0020. ; 114:4, s. 791-795
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Journal article (peer-reviewed)abstract
- Familial clustering of the precursor condition, monoclonal gammopathy of undetermined significance (MGUS) has been observed in case reports and in smaller studies. Using population-based data from Sweden, we identified 4458 MGUS patients, 17505 population-based controls, and first-degree relatives of patients (n = 14621) and controls (n = 58387) with the aim to assess risk of MGUS and lymphoproliferative malignancies among first-degree relatives of MGUS patients. Compared with relatives of controls, relatives of MGUS patients had increased risk of MGUS (relative risk [RR] = 2.8; 1.4-5.6), multiple myeloma (MM; RR = 2.9; 1.9-4.3), lymphoplasmacytic lymphoma/Waldenström macroglobulinemia (LPL/WM; RR = 4.0; 1.5-11), and chronic lymphocytic leukemia (CLL; RR = 2.0; 1.2-2.3). Relatives of patients with IgG/IgA MGUS had a 4.0-fold (1.7-9.2), 2.9-fold (1.7-4.9), and 20-fold (2.3-170) elevated risk of developing MGUS, MM, and LPL/WM, respectively. Relatives of IgM MGUS patients had 5.0-fold (1.1-23) increased CLL risk and nonsignificant excess MM and LPL/WM risks. The results were very similar when we assessed risk by type of first-degree relative, age at MGUS (above/below 65 years), or sex. Risk of non-Hodgkin lymphoma or Hodgkin lymphoma was not increased among MGUS relatives. Among first-degree relatives of a nationwide MGUS cohort, we found elevated risks of MGUS, MM, LPL/WM, and CLL, supporting a role for germline susceptibility genes, shared environmental influences, or an interaction between both.
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- Turesson, Ingemar, et al.
(author)
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Ascertainment and diagnostic accuracy for hematopoietic lymphoproliferative malignancies in Sweden 1964-2003.
- 2007
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In: International Journal of Cancer. - : Wiley. - 0020-7136 .- 1097-0215. ; 121:10, s. 2260-2266
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Journal article (peer-reviewed)abstract
- Population-based cancer registries are widely used to provide key information about cancer incidence, survival, determinants of progression and clues about pathogenesis. To substantially expand the limited data on diagnostic accuracy and completeness for lym-phoproliferative (LP) tumors in such databases, we conducted a retrospective investigation of close to 1,000 cases diagnosed during 4 decades in Sweden. We identified and reviewed medical records for 494 LP tumor patients reported to the population-based Swedish Cancer registry and 503 LP tumor patients identified from hospitals in Sweden among patients with LP tumors diagnosed during 1964-2003. The stratified samples were randomly selected from patients according to LP subtype, over 4 equal calendar periods, and among 6 selected hospitals of diverse size and from different geographic regions. We found 97.9% of reported LP tumor cases to fulfill current diagnostic criteria for having an LP tumor and observed 89.7% to have accurate LP tumor subtype. The overall completeness of non-Hodgkin lymphoma, Hodgkin lymphoma and multiple myeloma cases in the Cancer registry was 95-99% but was lower for the more indolent tumors, chronic lymphocytic leukemia (87.9%) and Waldenstrom's macroglobulinemia (68.1%). We observed increased overall under-ascertamment for patients diagnosed above age 80 (27%) and among individuals diagnosed before 1973 (12%). In conclusion the diagnostic accuracy and completeness for classically defined LP tumor entities in the Swedish Cancer registry is high. However, we found under-ascertainment of patients with indolent LP tumors, particularly among patients diagnosed at older ages, with early-stage disease and diagnosed in earlier years.
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