How “benign” is cutaneous mastocytosis? A Danish registry-based matched cohort study

Background: There 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 r...

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Main Authors: Line Kibsgaard, MD, PhD, Mette Deleuran, MD, Head of Department, Carsten Flohr, MD, Head of Department, Sinéad Langan, MD, Anne Braae Olesen, MD, PhD, Christian Vestergaard, MD, PhD
Format: Article
Language:English
Published: Elsevier 2020-09-01
Series:International Journal of Women's Dermatology
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2352647520300964
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spelling doaj-288737177ab54fe68aa80206f42c5a542020-11-25T03:42:30ZengElsevierInternational Journal of Women's Dermatology2352-64752020-09-0164294300How “benign” is cutaneous mastocytosis? A Danish registry-based matched cohort studyLine Kibsgaard, MD, PhD0Mette Deleuran, MD, Head of Department1Carsten Flohr, MD, Head of Department2Sinéad Langan, MD3Anne Braae Olesen, MD, PhD4Christian Vestergaard, MD, PhD5Department of Dermatology, Aarhus University Hospital, Aarhus, Denmark; Corresponding author.Department of Dermatology, Aarhus University Hospital, Aarhus, DenmarkUnit for Population-based Dermatology Research, St John’s Institute of Dermatology, Guy’s & St Thomas’ NHS Foundation Trust and Kings College London, London, United KingdomFaculty of Epidemiology & Population Health, London School of Hygiene and Tropical Medicine, London, United KingdomDepartment of Dermatology, Aarhus University Hospital, Aarhus, DenmarkDepartment of Dermatology, Aarhus University Hospital, Aarhus, DenmarkBackground: There 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. Objective: This study aimed to analyze the incidence and mortality of mastocytosis. 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. 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. Results: A 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. Conclusion: Based 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.http://www.sciencedirect.com/science/article/pii/S2352647520300964EpidemiologyMastocytosisComorbidity-adjusted analysesSurvival analysesDanish National Patient Registry
collection DOAJ
language English
format Article
sources DOAJ
author Line Kibsgaard, MD, PhD
Mette Deleuran, MD, Head of Department
Carsten Flohr, MD, Head of Department
Sinéad Langan, MD
Anne Braae Olesen, MD, PhD
Christian Vestergaard, MD, PhD
spellingShingle Line Kibsgaard, MD, PhD
Mette Deleuran, MD, Head of Department
Carsten Flohr, MD, Head of Department
Sinéad Langan, MD
Anne Braae Olesen, MD, PhD
Christian Vestergaard, MD, PhD
How “benign” is cutaneous mastocytosis? A Danish registry-based matched cohort study
International Journal of Women's Dermatology
Epidemiology
Mastocytosis
Comorbidity-adjusted analyses
Survival analyses
Danish National Patient Registry
author_facet Line Kibsgaard, MD, PhD
Mette Deleuran, MD, Head of Department
Carsten Flohr, MD, Head of Department
Sinéad Langan, MD
Anne Braae Olesen, MD, PhD
Christian Vestergaard, MD, PhD
author_sort Line Kibsgaard, MD, PhD
title How “benign” is cutaneous mastocytosis? A Danish registry-based matched cohort study
title_short How “benign” is cutaneous mastocytosis? A Danish registry-based matched cohort study
title_full How “benign” is cutaneous mastocytosis? A Danish registry-based matched cohort study
title_fullStr How “benign” is cutaneous mastocytosis? A Danish registry-based matched cohort study
title_full_unstemmed How “benign” is cutaneous mastocytosis? A Danish registry-based matched cohort study
title_sort how “benign” is cutaneous mastocytosis? a danish registry-based matched cohort study
publisher Elsevier
series International Journal of Women's Dermatology
issn 2352-6475
publishDate 2020-09-01
description Background: There 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. Objective: This study aimed to analyze the incidence and mortality of mastocytosis. 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. 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. Results: A 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. Conclusion: Based 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.
topic Epidemiology
Mastocytosis
Comorbidity-adjusted analyses
Survival analyses
Danish National Patient Registry
url http://www.sciencedirect.com/science/article/pii/S2352647520300964
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