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Hamostaseologie 2019; 39(03): 284-293
DOI: 10.1055/s-0038-1675354
Original Article
Georg Thieme Verlag KG Stuttgart · New York

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

Epidemiologie und Therapie der Hämophilie in Österreich—Update aus dem Österreichischen Hämophilie Register
Judit Rejtő
1   Clinical Division of Haematology and Haemostaseology, Department of Medicine I, Medical University of Vienna, Vienna, Austria
,
Sylvia Reitter-Pfoertner
1   Clinical Division of Haematology and Haemostaseology, Department of Medicine I, Medical University of Vienna, Vienna, Austria
,
Sylvia Kepa
1   Clinical Division of Haematology and Haemostaseology, Department of Medicine I, Medical University of Vienna, Vienna, Austria
,
Clemens Feistritzer
2   Department of Internal Medicine V – Haematology and Oncology, Medical University of Innsbruck, Innsbruck, Austria
,
Michael Grundbichler
3   IIIrd Medical Department, Paracelsus Medical University Salzburg, Salzburg Cancer Research Institute, Salzburg, Austria
,
Alexander Hörbst
4   eHealth Research and Innovation Unit, UMIT – University for Health Sciences, Medical Informatics and Technology, Hall in Tirol, Austria
,
Neil Jones
5   Department of Pediatrics and Adolescent Medicine, Paracelsus Private Medical University, Salzburg, Austria
,
Wolfgang Muntean
6   Department of General Pediatrics and Adolescent Medicine, Medical University of Graz, Graz, Austria
,
Peter Neumeister
7   Division of Haematology, Department of Internal Medicine, Medical University of Graz, Graz, Austria
,
Stefan Oberbichler
4   eHealth Research and Innovation Unit, UMIT – University for Health Sciences, Medical Informatics and Technology, Hall in Tirol, Austria
,
Gerhard Schuster
8   Austrian Red Cross, Blood Transfusion Service for Upper Austria, Linz, Austria
,
Rudolf Schwarz
9   Department of Paediatric and Adolescent Medicine, Kepler University Hospital Linz, Linz, Austria
,
Katharina Thom
10   Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria
,
Karl Zwiauer
11   Department of Paediatrics, Universitätsklinikum St. Pölten, Karl Landsteiner University of Health Sciences Austria, St. Pölten, Austria
,
Werner Streif
12   Department of Pediatrics, Medical University of Innsbruck, Innsbruck, Austria
,
Christoph Male
10   Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria
,
Ingrid Pabinger
1   Clinical Division of Haematology and Haemostaseology, Department of Medicine I, Medical University of Vienna, Vienna, Austria
› Author Affiliations
Further Information

Publication History

04 May 2018

17 September 2018

Publication Date:
12 November 2018 (online)

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Abstract

The 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.

Zusammenfassung

Das Österreichische Hämophilieregister sammelt epidemiologische Daten über Patienten mit Hämophilie, über die Behandlungsmodalitäten und über die möglichen Nebenwirkungen. Das Österreichische Hämophilie Register erfasst mehr als 85% der zu erwartenden Anzahl von Hämophilen in Österreich. Im Register sind 753 Patienten erfasst, von denen 84.3% (635) Hämophilie A und 15.7% (118) Hämophilie B haben. Das mediane Alter ist 34 Jahre (1–93). Es haben 39.0% (294) eine schwere, 11.3% (85) eine mittelschwere und 49.4% (372) eine leichte Hämophilie. Von den Patienten mit schwerer Hämophilie wurden 38.4% (113) mit HCV infiziert und 12.6% (37) sind HIV positiv. Insgesamt haben 10.6% (67) aller Patienten mit Hämophilie A und 1.7% (2) aller Patienten mit Hämophilie B einen Hemmkörper in ihrer Anamnese. Von den Patienten mit schwerer Hämophilie erhalten 68.4% (201) eine Prophylaxe und 28.6% (84) eine Bedarfsbehandlung. Von den Patienten mit schwerer Hämophilie erhalten 65.0% (191) ein rekombinantes Produkt. Zusammenfassend erhalten die meisten Patienten mit schwerer Hämophilie eine Prophylaxe. HCV und HIV Infektionen sind immer noch von großer Bedeutung für die österreichischen Hämophilie-Patienten.

Keywords

Austrian Haemophilia Registry - hepatitis C virus - congenital bleeding disorder

Schlüsselwörter

Österreichisches Hämophilieregister - Hepatitis C Virus - angeborene Blutungsstörung
 

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