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. 2020 May 22:12:497-508.
doi: 10.2147/CLEP.S250250. eCollection 2020.

Increasing Incidence and Prevalence of Acquired Hemolytic Anemias in Denmark, 1980-2016

Dennis Lund Hansen  1   2 , Sören Möller  1   3 , Kjeld Andersen  1   4 , David Gaist  1   5 , Henrik Frederiksen  1   2
Affiliations

Increasing Incidence and Prevalence of Acquired Hemolytic Anemias in Denmark, 1980-2016

Dennis Lund Hansen et al. Clin Epidemiol. .

Abstract

Background: Acquired hemolytic disorders-autoimmune hemolytic anemia (AIHA), cold agglutinin disease (CAD), paroxysmal nocturnal hemoglobinuria (PNH), drug-induced hemolysis (DIHA), and acquired hemolysis not otherwise specified (AHNOS)-are considered rare. Despite their potentially major health implications, data regarding their incidence and prevalence are scarce.

Methods: To fill this gap we collected data regarding all patients with acquired hemolytic disorder diagnoses in 1977-2016 from the Danish National Patient Register. These data were linked with vital and migration status information from the Danish Civil Registration System. From these data combined with annual demographic data for the background population, we calculated age- and sex-specific incidence rates and prevalence proportions of acquired hemolytic disorders for specified time periods.

Results: Our analysis included 5868 patients with acquired hemolytic disorders (2715 with AIHA, 112 CAD, 397 DIHA, 116 PNH, and 2154 AHNOS). The incidence rates per 100 000 person-years in 1980-1993 and 2008-2016 were 0.81 and 1.77 for AIHA, 0.31 and 0.12 for DIHA, and 0.04 and 0.08 for PNH, respectively. The 2008-2016 CAD incidence rate was 0.18/100 000 person-years, CAD diagnosis code was not defined before 1994. All incidence rates increased with age. The prevalence proportion per 100 000 persons in 1980 and 2015 was 2.52 and 17.01 for AIHA, 0.80 and 1.50 for DIHA, and 0.18 and 1.04 for PNH. CAD prevalence in 2015 was 1.04/100 000 persons.

Conclusion: Acquired hemolytic anemia incidence rates and prevalence proportions with the exception of DIHA are markedly increasing.

Keywords: autoimmune hemolytic anemia; cold agglutinin disease; drug-induced hemolytic anemia; incidence; paroxysmal nocturnal hemoglobinuria; prevalence.

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Conflict of interest statement

DLH declares that as part of the funding for the PhD fellowship, this study received funding from the University of Southern Denmark, The Region of Southern Denmark, and study grants from Alexion Pharma Nordic AB, Novartis Healthcare and The A. P. Møller and Chastine Mc-Kinney Møller Foundation. The remaining authors declare no competing financial interests. Beyond this, none of the authors have any other conflicts of interest with the study.

Figures

Figure 1
Figure 1
Prevalence of acquired hemolysis in Denmark, 1980–2016. Notes: The overall prevalence proportion with 95% confidence intervals for all acquired hemolytic diseases, calculated on 1st of January each year, using census data as the denominator. The 95% confidence intervals were calculated using the Clopper–Pearson method. CAD diagnosis was not defined in the ICD before 1994. Data were based on a national cohort of patients from Denmark diagnosed in 1980–2016. Abbreviations: AIHA, autoimmune hemolytic anemia; CAD, cold agglutinin disease; CI, confidence interval; NOS, not otherwise specified; PNH, paroxysmal nocturnal hemoglobinuria.
See this image and copyright information in PMC

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