https://orcid.org/0000-0002-4478-1297
![Sample our Bioscience journals, sign in here to start your access, Latest two full volumes FREE to you for 14 days]({"type":"image" , "src" : "/sda/5925/TOC-Subject-Sample-2021-Bioscience.jpg"})
Abstract
Background
Congenital red blood cell (RBC) disorders, such as hemoglobinopathies, are frequent worldwide but with large geographical variation. Growing migration has increased the number of patients with RBC disorders in formerly low prevalence countries, eg, Denmark. However, accurate prevalences are unknown.
Methods
Patients with a registered diagnosis of congenital hemolysis in the Danish National Patient Register between 1977 and 2016 were linked to a national laboratory database of RBC disorders and the Danish civil registration system. We calculate annual age- and sex-specific prevalences of the congenital hemolytic disorders from 2000 to 2016.
Results
Prevalences of all subtypes of congenital hemolytic disorders increased during the study period. The prevalence of hereditary spherocytosis increased 1.73 times between 2000 and 2015, from 10.2/105 persons to 17.7/105 persons. Alpha thalassemia trait had a prevalence of 0.5/105 persons in 2000, but increased 41 times to 19.2/105 persons in 2015. Beta thalassemia minor increased eightfold from 4.5/105 persons in 2000 to 34.9/105 persons in 2015. Likewise, sickle cell trait increased 11 times from 0.7/105 persons in 2000 to 8.1/105 persons in 2015, whereas sickle cell disease increased from 0.5/105 persons to 2.7/105 persons in 2015, a fivefold increase.
Conclusion
The prevalence of congenital RBC disorders in Denmark is increasing. The hemoglobinopathy traits now have prevalences as high as hereditary spherocytosis. These estimates of congenital hemolytic disorders in Denmark emphasize that inborn hemoglobin disorders are a public health concern, even in some formerly low prevalence countries.
Acknowledgments
The authors would like to thank Cathrine Fox Maule at Statistics Denmark for help with defining and hosting data. The authors also thank the University of Southern Denmark, The Region of Southern Denmark, Alexion Pharma Nordic AB, The A. P. Møller and Chastine Mc-Kinney Møller Foundation, and Novartis Healthcare for study grants.
Author Contributions
DLH, HF, DG, and KA conceived the study. AG and JP provided laboratory data and insight into laboratory diagnostics. All authors took part in planning the analyses. DLH performed the analyses, aided by SM. DLH wrote first draft, and all authors participated in writing subsequent drafts. All authors made substantial contributions to conception and design, acquisition of data, or analysis and interpretation of data; took part in drafting the article or revising it critically for important intellectual content; gave final approval of the version to be published; and agreed to be accountable for all aspects of the work.
Disclosure
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. AG reports personal fees from Agios, Bluebird, Celgene, Novartis, and Novo Nordisk; grants from Alexion, outside the submitted work. The authors report no other conflicts of interest in this work.