Prevalence and incidence of mastocytosis in adults: a Danish nationwide register study

Introduction: Mastocytosis is a group of rare heterogeneous diseases with a prevalence previously found to be 10–23 per 100,000 persons. More awareness and improvements in the diagnostic methods in later years have led to more patients being diagnosed. Here, we set out to present the prevalence and incidence rate of mastocytosis among the adult Danish population. By merging data from the Danish National Patient Register, the Danish Pathology Register and the Danish Cancer Register we included all adult patients (≥ 18 years) diagnosed with mastocytosis in Denmark prior to 2022. A cohort of 1,594 patients with mastocytosis was identified. The prevalence of mastocytosis was 27.43 per 100,000 persons (95% confidence interval [CI]: 25.95–28.96) as of January 1, 2022, and the 25-year average incidence rate between 1997 and 2021 was 1.21 per 100,000 persons (95%CI: 1.02–1.40) with an increasing incidence rate since 2002. We found a higher prevalence of mastocytosis among adults in the Danish population than previously reported, and an increasing incidence rate during the last 20 years. Increased awareness of the disease and better diagnostic methods most likely contributed to this.