2014PubMedOpen access

The European Haemophilia Network (EUHANET).

Michael Makris, Gabriele Calizzani, Kathelijn Fischer, Alexander Gatt, Estelle Gilman, Robert E. Hollingsworth, Thierry Lambert, Riitta Lassila, Pier Mannuccio Mannucci, Flora Peyvandi, Jerzy Windyga

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Abstract

Haemophilia A and B are rare inherited bleeding disorders due to reduced factor VIII (FVIII) or factor IX (FIX) activity, occurring in 1 in 10,000 and 1 in 50,000 of the population respectively1,2. Several other bleeding disorders such as deficiencies of factors I, II, V, VII, X, XI and XIII are even rarer. When the deficiency is severe these disorders present with spontaneous bleeding whilst for milder disorders traumatic bleeding is observed3. When bleeding occurs patients are treated with clotting factor concentrates which can be plasma derived or recombinant. Individuals with inherited bleeding disorders are cared for in Haemophilia Centres. There is major disparity in patient access to Haemophilia Centres throughout Europe4, although it must be appreciated that this publication was based on data from the individual country patient organisations and was not based on national registries. There is a need for harmonisation of the data available in care centres, national data sources and patient organisations and these activities are planned to be improved by the European Haemophilia Network (EUHANET) project. There is also variation in the number of Haemophilia Centres within European countries and whilst in some there are over 80 Haemophilia Centres in others there is only a single centre. The use of clotting factor concentrates has been associated with major adverse effects. Patients treated with plasma derived clotting concentrates prior to 1985 had an almost 100% risk of being infected with hepatitis C (HCV) and a 30–60% risk of Human Immunodeficiency Virus (HIV) infection5. Although the HCV/HIV viruses were eliminated following the introduction of viral inactivation, improvements in diagnostic tools used for the biological qualification of blood donations and the use of recombinant concentrates, other adverse events such as alloantibodies to FVIII or FIX remain6. The rarity of these disorders makes it difficult to determine the precise frequency of the adverse events because large numbers of patients are required which are not available in single centres. To overcome this problem, the European Haemophilia Safety Surveillance (EUHASS) system was set up in 20087. This was a collaboration of over 70 European Haemophilia Centres in 26 countries which agreed to prospectively report adverse events occurring in their patients. The project was funded by the European Commission with pharmaceutical industry support. The EUHASS project demonstrated the willingness of these Haemophilia Centres to work together so the EUHANET was set up. This is a collaboration in four separate areas, the certification of Haemophilia Centres, the set up of a haemophilia website, the expansion of the EUHASS system and the establishment of a prospective project on afibrinogenemia and FXIII deficiency.

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What this paper is about

Haemophilia A and B are rare inherited bleeding disorders due to reduced factor VIII (FVIII) or factor IX (FIX) activity, occurring in 1 in 10,000 and 1 in 50,000 of the population respectively1,2. Several other bleeding disorders such as deficiencies of factors I, II, V, VII, X, XI and XIII are even rarer. When the deficiency is severe these disorders present with spontaneous bleeding whilst for milder disorders traumatic bleeding is observed3. When bleeding occurs patients are treated with clotting factor concentrates which can be plasma derived or recombinant. Individuals with inherited bleeding disorders are cared for in Haemophilia Centres. There is major disparity in patient access to Haemophilia Centres throughout Europe4, although it must be appreciated that this publication was based on data from the individual country patient organisations and was not based on national registries. There is a need for harmonisation of the data available in care centres, national data sources and patient organisations and these activities are planned to be improved by the European Haemophilia Network (EUHANET) project. There is also variation in the number of Haemophilia Centres within European countries and whilst in some there are over 80 Haemophilia Centres in others there is only a single centre. The use of clotting factor concentrates has been associated with major adverse effects. Patients treated with plasma derived clotting concentrates prior to 1985 had an almost 100% risk of being infected with hepatitis C (HCV) and a 30–60% risk of Human Immunodeficiency Virus (HIV) infection5. Although the HCV/HIV viruses were eliminated following the introduction of viral inactivation, improvements in diagnostic tools used for the biological qualification of blood donations and the use of recombinant concentrates, other adverse events such as alloantibodies to FVIII or FIX remain6. The rarity of these disorders makes it difficult to determine the precise frequency of the adverse events because large numbers of patients are required which are not available in single centres. To overcome this problem, the European Haemophilia Safety Surveillance (EUHASS) system was set up in 20087. This was a collaboration of over 70 European Haemophilia Centres in 26 countries which agreed to prospectively report adverse events occurring in their patients. The project was funded by the European Commission with pharmaceutical industry support. The EUHASS project demonstrated the willingness of these Haemophilia Centres to work together so the EUHANET was set up. This is a collaboration in four separate areas, the certification of Haemophilia Centres, the set up of a haemophilia website, the expansion of the EUHASS system and the establishment of a prospective project on afibrinogenemia and FXIII deficiency.

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Available abstract

Haemophilia A and B are rare inherited bleeding disorders due to reduced factor VIII (FVIII) or factor IX (FIX) activity, occurring in 1 in 10,000 and 1 in 50,000 of the population respectively1,2. Several other bleeding disorders such as deficiencies of factors I, II, V, VII, X, XI and XIII are even rarer. When the deficiency is severe these disorders present with spontaneous bleeding whilst for milder disorders traumatic bleeding is observed3. When bleeding occurs patients are treated with clotting factor concentrates which can be plasma derived or recombinant. Individuals with inherited bleeding disorders are cared for in Haemophilia Centres. There is major disparity in patient access to Haemophilia Centres throughout Europe4, although it must be appreciated that this publication was based on data from the individual country patient organisations and was not based on national registries. There is a need for harmonisation of the data available in care centres, national data sources and patient organisations and these activities are planned to be improved by the European Haemophilia Network (EUHANET) project. There is also variation in the number of Haemophilia Centres within European countries and whilst in some there are over 80 Haemophilia Centres in others there is only a single centre. The use of clotting factor concentrates has been associated with major adverse effects. Patients treated with plasma derived clotting concentrates prior to 1985 had an almost 100% risk of being infected with hepatitis C (HCV) and a 30–60% risk of Human Immunodeficiency Virus (HIV) infection5. Although the HCV/HIV viruses were eliminated following the introduction of viral inactivation, improvements in diagnostic tools used for the biological qualification of blood donations and the use of recombinant concentrates, other adverse events such as alloantibodies to FVIII or FIX remain6. The rarity of these disorders makes it difficult to determine the precise frequency of the adverse events because large numbers of patients are required which are not available in single centres. To overcome this problem, the European Haemophilia Safety Surveillance (EUHASS) system was set up in 20087. This was a collaboration of over 70 European Haemophilia Centres in 26 countries which agreed to prospectively report adverse events occurring in their patients. The project was funded by the European Commission with pharmaceutical industry support. The EUHASS project demonstrated the willingness of these Haemophilia Centres to work together so the EUHANET was set up. This is a collaboration in four separate areas, the certification of Haemophilia Centres, the set up of a haemophilia website, the expansion of the EUHASS system and the establishment of a prospective project on afibrinogenemia and FXIII deficiency.

Key concepts: Haemophilia, Clotting factor, Haemophilia A, Medicine, Haemophilia B, Factor IX, Pediatrics, Population

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