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

Acta Oncol

Department of Medical Epidemiology and Biostatistics, Karolinska Institutet, Stockholm, Sweden; Regional Cancer Center, Central Sweden, Uppsala, Sweden.

Published: February 2024

AI Article Synopsis

  • - Researchers studied the incidence, prevalence, survival rates, and comorbidities of adult mastocytosis patients in Sweden from 2001 to 2018 using national health registries.
  • - They identified 2,040 cases of mastocytosis, with an annual incidence of 1.56 per 100,000 and a prevalence of 23.9 per 100,000, finding that patients had a higher burden of other diseases and lower overall survival compared to matched controls.
  • - The study concluded that mastocytosis is more common than previously thought in other settings and highlighted the need for better diagnostics, while acknowledging limitations like underreporting of cases.

Article 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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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11332469PMC
http://dx.doi.org/10.2340/1651-226X.2024.31406DOI Listing

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