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Prevalence of Congenital Hemolytic Disorders in Denmark, 2000–2016

Authors Hansen DL, Glenthøj A, Möller S, Biemond BJ, Andersen K, Gaist D, Petersen J, Frederiksen H

Received 18 February 2020

Accepted for publication 7 April 2020

Published 21 May 2020 Volume 2020:12 Pages 485—495


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 2

Editor who approved publication: Dr Eyal Cohen

Dennis Lund Hansen,1,2 Andreas Glenthøj,3 Sören Möller,1,4 Bart J Biemond,5 Kjeld Andersen,1,6 David Gaist,1,7 Jesper Petersen,3 Henrik Frederiksen1,2

1Department of Clinical Research, University of Southern Denmark, Odense, Denmark; 2Department of Hematology, Odense University Hospital, Odense, Denmark; 3Department of Hematology, Center for Hemoglobinopathies, Herlev and Gentofte Hospital, Herlev, Denmark; 4OPEN, Odense University Hospital, Odense, Denmark; 5Department of Hematology, Amsterdam University Medical Centre’s, Amsterdam, Netherlands; 6Department of Mental Health - Odense, Region of Southern Denmark, Odense, Denmark; 7Neurology Research Unit, Department of Neurology, Odense University Hospital, Odense, Denmark

Correspondence: Dennis Lund Hansen Email

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.

Keywords: hemolytic anemia, thalassemia, sickle cell disease, hereditary spherocytosis, hemoglobinopathies, sickle cell anemia

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