Abstract
BackgroundThere are limited estimates of the incidence rates (IRs) of mastocytosis, and only a few studies have addressed the long-term consequences of living with these diagnoses. Previous reports have shown that systemic mastocytosis is associated with leukemic transformations and an increased risk of death as opposed to cutaneous mastocytosis (CM) and indolent systemic mastocytosis (ISM), which have benign diagnoses with life expectancy rates similar to those of the background population.ObjectiveThis study aimed to analyze the incidence and mortality of mastocytosis.MethodsA population-based matched cohort study of patients with mastocytosis between 1 January 1, 1977 and 31 December 31, 2014 was identified from the Danish National Health Registries. IRs of CM, ISM, and pediatric mastocytosis were highlighted. Survival estimates were compared with those of a healthy background population, using a Cox proportional hazard model.ResultsA total of 1461 patients with mastocytosis were identified. The annual IR of overall mastocytosis was 1.1 per 100,000 person years (95% confidence interval [CI], 1.0–1.2). Among children, the IR was 1.8 per 100,000 person years (95% CI, 1.6–2.1). The prevalence of any comorbidity was twice as high among patients with mastocytosis compared with the population without mastocytosis (odds ratio: 2.1; 95% CI, 1.8–2.5). The Charlson Comorbidity Index–adjusted mortality among adult patients with mastocytosis was HRCutaneous Mastocytosis 1.2 (95% CI, 0.8–1.9), HRIndolent Systemic Mastocytosis 1.9 (95% CI 1.4–2.5), and HRSystemic Mastocytosis 4.2 (95%, CI 1.9–9.4), respectively.ConclusionBased on an entire nation, with free health care at the point of access, we estimated an annual IR of mastocytosis and its subgroups. We discovered that patients with ISM had an increased risk of death compared with the general population. Our data supported the overall benign nature of CM diagnosed after age 2 years.
Highlights
Mastocytosis is a heterogeneous group of rare mast cell disorders that may affect both children and adults
25 patients were excluded, either because the inclusion date came after the registered date of death (n = 1) or because the ICD10 diagnoses were registered before the official date of the ICD10 implementation (n = 24)
Based on historical Danish registry data, we discovered a frequency of 65 new Danish cases of mastocytosis per year
Summary
Mastocytosis is a heterogeneous group of rare mast cell disorders that may affect both children and adults. Aggressive subtypes of mastocytosis are reported in infants and in patients aged 60 years (Bodemer et al, 2010; Escribano et al, 2009). Previous reports have shown that systemic mastocytosis is associated with leukemic transformations and an increased risk of death as opposed to cutaneous mastocytosis (CM) and indolent systemic mastocytosis (ISM), which have benign diagnoses with life expectancy rates similar to those of the background population. Methods: A population-based matched cohort study of patients with mastocytosis between 1 January 1, 1977 and 31 December 31, 2014 was identified from the Danish National Health Registries. We discovered that patients with ISM had an increased risk of death compared with the general population.
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