Epidemiology and Treatment of Patients with Haemophilia in Austria—Update from the Austrian Haemophilia Registry

Author:

Rejtő Judit1,Reitter-Pfoertner Sylvia1,Kepa Sylvia1,Feistritzer Clemens2,Grundbichler Michael3,Hörbst Alexander4,Jones Neil5,Muntean Wolfgang6,Neumeister Peter7,Oberbichler Stefan4,Schuster Gerhard8,Schwarz Rudolf9,Thom Katharina10,Zwiauer Karl11,Streif Werner12,Male Christoph10,Pabinger Ingrid1

Affiliation:

1. Clinical Division of Haematology and Haemostaseology, Department of Medicine I, Medical University of Vienna, Vienna, Austria

2. Department of Internal Medicine V – Haematology and Oncology, Medical University of Innsbruck, Innsbruck, Austria

3. IIIrd Medical Department, Paracelsus Medical University Salzburg, Salzburg Cancer Research Institute, Salzburg, Austria

4. eHealth Research and Innovation Unit, UMIT – University for Health Sciences, Medical Informatics and Technology, Hall in Tirol, Austria

5. Department of Pediatrics and Adolescent Medicine, Paracelsus Private Medical University, Salzburg, Austria

6. Department of General Pediatrics and Adolescent Medicine, Medical University of Graz, Graz, Austria

7. Division of Haematology, Department of Internal Medicine, Medical University of Graz, Graz, Austria

8. Austrian Red Cross, Blood Transfusion Service for Upper Austria, Linz, Austria

9. Department of Paediatric and Adolescent Medicine, Kepler University Hospital Linz, Linz, Austria

10. Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria

11. Department of Paediatrics, Universitätsklinikum St. Pölten, Karl Landsteiner University of Health Sciences Austria, St. Pölten, Austria

12. Department of Pediatrics, Medical University of Innsbruck, Innsbruck, Austria

Abstract

AbstractThe Austrian Haemophilia Registry collects epidemiological data on patients with haemophilia, on treatment modalities and potential side effects. The Registry covers more than 85% of the assumed total number of haemophilia patients in Austria. This report summarizes data on 753 patients: 84.3% (635) have haemophilia A and 15.7% (118) have haemophilia B. Patients' median age is 34 years (range: 1–93 years). Of the total cohort, 39.0% (294) patients have severe haemophilia, 11.3% (85) moderate haemophilia, and 49.4% (372) mild haemophilia. Of the patients with severe haemophilia, 38.4% (113) have been infected with hepatitis C virus (HCV) and 12.6% (37) are human immunodeficiency virus (HIV) positive. Overall, 10.6% (67) of patients with haemophilia A and 1.7% (2) of those with haemophilia B have had an inhibitor in their history. Among patients with severe haemophilia, 68.4% (201) receive prophylaxis and 28.6% (84) receive on-demand therapy. There are 65.0% (191) patients with severe haemophilia who are treated with recombinant products. In conclusion, most patients with severe haemophilia receive prophylactic treatment. HCV and HIV infections are still important issues in the Austrian haemophilia population.

Publisher

Georg Thieme Verlag KG

Subject

Hematology

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