Epidemiology of mastocytosis: a population-based study (Sweden).
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ABSTRACT: Background
Mastocytosis is a disease characterized by accumulation of aberrant mast cells and mediator-related symptoms and is divided into systemic mastocytosis (SM) and cutaneous mastocytosis (CM). The epidemiology of mastocytosis remains incompletely understood.Objective
To estimate the incidence, prevalence, overall survival (OS) and burden of comorbidities in adult mastocytosis patients identified in Swedish population-based registries.Methods
Individuals (≥ 20 years of age) with a mastocytosis diagnosis in the National Patient Register (NPR) and/or the Swedish Cancer Register (SCR) between 2001 and 2018, were identified. In a matched cohort design, for each case five randomly selected mastocytosis-free comparators matched on age, sex, and county of residence were chosen from the Population Register. The Kaplan-Meier method was used to compare OS between individuals with mastocytosis and comparators. Information on concomitant disease at baseline was assessed by use of the Charlson Comorbidity Index (CCI).Results
We identified 2,040 adults with a mastocytosis diagnosis yielding an annual incidence of 1.56 per 100,000 (95% CI 1.29-1.87) and a prevalence of 23.9 per 100,000 (95% CI 22.8-25.0). The comorbidity burden was higher, and the OS lower, in patients with mastocytosis compared to comparators.Interpretation
We found a higher incidence and prevalence of mastocytosis compared to assessments in other settings and confirmed that the prognosis generally is favorable. Of special note was evidence of a higher comorbidity burden in mastocytosis patients compared to the background population.Limitations
Underreporting and inconsistencies in the use of diagnostic codes.
SUBMITTER: Bergstrom A
PROVIDER: S-EPMC11332469 | biostudies-literature | 2024 Feb
REPOSITORIES: biostudies-literature
Publications
Epidemiology of mastocytosis: a population-based study (Sweden).
Bergström Anna A Hägglund Hans H Berglund Anders A Nilsson Gunnar G Lambe Mats M
Acta oncologica (Stockholm, Sweden) 20240221
<h4>Background</h4>Mastocytosis is a disease characterized by accumulation of aberrant mast cells and mediator-related symptoms and is divided into systemic mastocytosis (SM) and cutaneous mastocytosis (CM). The epidemiology of mastocytosis remains incompletely understood.<h4>Objective</h4>To estimate the incidence, prevalence, overall survival (OS) and burden of comorbidities in adult mastocytosis patients identified in Swedish population-based registries.<h4>Methods</h4>Individuals (≥ 20 years ...[more]
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