title: Risk of solid cancer, cardiovascular disease, anaphylaxis, osteoporosis and fractures in patients with systemic mastocytosis: A nationwide population-based study.
abstract: In patients with systemic mastocytosis (SM), several aspects of morbidity remain poorly understood. We assessed the risk of solid cancers, cardiovascular disease, anaphylaxis, osteoporosis, and fractures in SM patients. Using Danish medical registries, we conducted a nationwide population-based cohort study including 687 adult (≥15 years) SM patients diagnosed during 1997-2012. A comparison cohort of 68,700 subjects from the general Danish population who were alive and without SM at the given SM subject's diagnosis were age- and gender-matched. Outcomes were a new diagnosis of solid cancer, venous thromboembolism (VTE), myocardial infarction (MI), stroke, anaphylaxis, osteoporosis, or fracture. For solid cancers the hazard ratio (HR) was 2.4 (95% confidence interval [CI] 1.9-2.8) with a 10-year absolute risk (AR) in the SM-cohort of 12.6% (95% CI 9.4-16.3). Specifically, we found a HR of 7.5 (95% CI 4.4-13.0) for melanoma and a HR of 2.5 (95% CI 1.7-3.5) for non-melanoma skin cancers (NMSCs). For VTE we found a HR of 1.9 (95% CI 1.2-3.0), with a 10-year AR of 3.9% (95% CI 2.3-6.1); for MI a nonsignificant increased HR of 1.4 (95% CI 0.9-2.3), with a 10-year AR of 1.8% (95% CI 0.9-3.2); and for stroke a HR of 1.6 (95% CI 1.1-2.3) with a 10-year AR of 4.6% (95% CI 2.8-6.9). The HR for anaphylaxis was 7.2 (95% CI 5.3-9.9), and the 10-year AR was 3.1% (95% CI 1.9-4.9). For osteoporosis the HR was 3.6 (95% CI 2.7-4.6) with a 10-year AR of 7.2% (95% CI 5.2-9.8). For fractures the HR was 1.2 (95% CI 0.9-1.6) and the 10-year AR was 5.9% (95% CI 3.9-8.4). SM patients are at increased risk of solid cancers - especially melanoma and NMSC-and cardiovascular disease. The risk of anaphylaxis and osteoporosis is clearly increased in SM, though absolute risk was low in this population-based study. The fracture-risk was only slightly increased. Am. J. Hematol. 91:1069-1075, 2016. © 2016 Wiley Periodicals, Inc.
authors.affiliation: Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark. sigurd.broesby-olsen@rsyd.dk.|Mastocytosis Centre, Odense University Hospital (MastOUH), Odense, Denmark. sigurd.broesby-olsen@rsyd.dk.
authors.forename: Sigurd
authors.initials: S
authors.lastname: Broesby-Olsen
authors.affiliation: Department of Clinical Epidemiology, Aarhus University Hospital, Denmark.
authors.forename: Dóra Körmendiné
authors.initials: DK
authors.lastname: Farkas
authors.affiliation: Mastocytosis Centre, Odense University Hospital (MastOUH), Odense, Denmark.|Department of Haematology, Odense University Hospital, Odense, Denmark.
authors.forename: Hanne
authors.initials: H
authors.lastname: Vestergaard
authors.affiliation: Mastocytosis Centre, Odense University Hospital (MastOUH), Odense, Denmark.|Department of Endocrinology, Odense University Hospital, Odense, Denmark.
authors.forename: Anne Pernille
authors.initials: AP
authors.lastname: Hermann
authors.affiliation: Mastocytosis Centre, Odense University Hospital (MastOUH), Odense, Denmark.|Department of Pathology, Odense University Hospital, Odense, Denmark.
authors.forename: Michael Boe
authors.initials: MB
authors.lastname: Møller
authors.affiliation: Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark.|Mastocytosis Centre, Odense University Hospital (MastOUH), Odense, Denmark.
authors.forename: Charlotte Gotthard
authors.initials: CG
authors.lastname: Mortz
authors.affiliation: Mastocytosis Centre, Odense University Hospital (MastOUH), Odense, Denmark.|Department of Pathology, Odense University Hospital, Odense, Denmark.
authors.forename: Thomas Kielsgaard
authors.initials: TK
authors.lastname: Kristensen
authors.affiliation: Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark.|Mastocytosis Centre, Odense University Hospital (MastOUH), Odense, Denmark.
authors.forename: Carsten
authors.initials: C
authors.lastname: Bindslev-Jensen
authors.affiliation: Department of Clinical Epidemiology, Aarhus University Hospital, Denmark.
authors.forename: Henrik Toft
authors.initials: HT
authors.lastname: Sørensen
authors.affiliation: Mastocytosis Centre, Odense University Hospital (MastOUH), Odense, Denmark.|Department of Clinical Epidemiology, Aarhus University Hospital, Denmark.|Department of Haematology, Odense University Hospital, Odense, Denmark.
authors.forename: Henrik
authors.initials: H
authors.lastname: Frederiksen
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000707:Anaphylaxis / Q000209:etiology; D002318:Cardiovascular Diseases / Q000209:etiology; D015331:Cohort Studies; D050723:Fractures, Bone / Q000209:etiology; D006801:Humans; D034721:Mastocytosis, Systemic / Q000150:complications* / Q000453:epidemiology*; D008875:Middle Aged; D009369:Neoplasms / Q000209:etiology; D009426:Netherlands / Q000453:epidemiology; D010024:Osteoporosis / Q000209:etiology; D016016:Proportional Hazards Models; D012042:Registries; D018570:Risk Assessment; D055815:Young Adult