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. 2024 Feb 21:63:44-50.
doi: 10.2340/1651-226X.2024.31406.

Epidemiology of mastocytosis: a population-based study (Sweden)

Anna Bergström  1 , Hans Hägglund  2 , Anders Berglund  3 , Gunnar Nilsson  4 , Mats Lambe  5
Affiliations

Epidemiology of mastocytosis: a population-based study (Sweden)

Anna Bergström et al. Acta Oncol. .

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.

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Conflict of interest statement

None declared

Figures

Figure 1
Figure 1
Overall survival in individuals with a mastocytosis diagnosis and comparators (controls) (A) and by mastocytosis subtype (B).
See this image and copyright information in PMC

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