Contributions
Abstract: O05
Type: Oral presentation
Session Topic: Inherited and acquired coagulation disorders
Presentation during EHA Scientific Conference on Bleeding Disorders:
On Thursday, September 15, 2016 from 09:00 - 10:30
Location: Rubí + Zafir
Background
The Austrian Haemophilia Registry is a web-based patient registry, which was initiated in 2007.
Aims
The primary aim of the Registry is to assess the frequency and the demographic characteristics of patients with congenital bleeding disorders. Secondary aims are to evaluate the treatment modalities and the potential side effects.
Methods
The Registry consists of three parts – part 1 and 2 containing data for quality control and assurance (this data is entered for all known patients); part 3 containing more detailed, patient-specific data (this data is only entered upon written informed consent of each patient). The presented data covers all patients who are treated at the eight Austrian haemophilia treatment centers. Summarized data are presented as percentage values or median and range, as appropriate.
Results
The total number of haemophiliac patients included in the Registry at the end of January 2016 was 753; thereof 635 patients (84 %) suffer from haemophilia A (HA), and 118 (16 %) have haemophilia B (HB).
Patients have a median age of 34 years (range: 1-93 years). Children (under the age of 18 years) represent 20 % and adults represent 80 % of the population.
In the study population, 39 % have severe (defined as factor VIII or IX levels < 1 %), 11 % have moderate (factor levels of 1-5 %) and 50 % have mild (factor levels of 5-50 %) haemophilia.
The age at diagnosis was available in 84 % of the patients. Patients who suffer from the severe form of the disease are typically diagnosed shortly after the first year of life, whereas non-severe patients were most likely to be diagnosed after the age of 4 years (with a median of 7 years of age).
Data is available on the treatment modalities of 94 % of the patients included in the database: prophylaxis is applied in 71 % of the patients with severe haemophilia, whereas 29 % receive on demand treatment. The number of severe haemophiliacs on prophylaxis was higher among children (91 %) than among adults (62 %). Regarding the type of product, 71 % of all patients with severe haemophilia have a recombinant product. In 72 % of all severe HA patients, a recombinant product is used, whereas this percentage is only 62.5 % in severe HB patients. Data on the viral status is available of 71.4 % of the patients with severe haemophilia. Overall, 13 % of all patients with severe HA are infected with HIV and 37 % are HCV-positive; a co-infection with both, HIV and HCV, has been confirmed in 10.7 %. Severe HB patients were HIV-positive in 4 %, HCV-positive in 37.5 % and in 4 % a co-infection with HIV and HCV was present. Currently, 3.6 % of all HA and 7.8 % of the severe HA patients have an inhibitor – 52 % of all HA patients with inhibitor and 57 % of the severe HA patients with inhibitor have high-titer inhibitor (> 5.0 Bethesda Units (BU)/ml). Presently 0.8 % of all our HB patients and 4 % of severe HB patients have a low-titer inhibitor (< 5.0 BU/ml).
Conclusion
The Austrian Haemophilia Registry enables us to obtain epidemiological data on haemophilia in Austria. The Registry also supports us in the effective planning of our scientific projects concerning bleeding disorders.
References
1. Reitter, S. et al, Wien Klin Wochenschr 2009; 121: 196–201
Abstract: O05
Type: Oral presentation
Session Topic: Inherited and acquired coagulation disorders
Presentation during EHA Scientific Conference on Bleeding Disorders:
On Thursday, September 15, 2016 from 09:00 - 10:30
Location: Rubí + Zafir
Background
The Austrian Haemophilia Registry is a web-based patient registry, which was initiated in 2007.
Aims
The primary aim of the Registry is to assess the frequency and the demographic characteristics of patients with congenital bleeding disorders. Secondary aims are to evaluate the treatment modalities and the potential side effects.
Methods
The Registry consists of three parts – part 1 and 2 containing data for quality control and assurance (this data is entered for all known patients); part 3 containing more detailed, patient-specific data (this data is only entered upon written informed consent of each patient). The presented data covers all patients who are treated at the eight Austrian haemophilia treatment centers. Summarized data are presented as percentage values or median and range, as appropriate.
Results
The total number of haemophiliac patients included in the Registry at the end of January 2016 was 753; thereof 635 patients (84 %) suffer from haemophilia A (HA), and 118 (16 %) have haemophilia B (HB).
Patients have a median age of 34 years (range: 1-93 years). Children (under the age of 18 years) represent 20 % and adults represent 80 % of the population.
In the study population, 39 % have severe (defined as factor VIII or IX levels < 1 %), 11 % have moderate (factor levels of 1-5 %) and 50 % have mild (factor levels of 5-50 %) haemophilia.
The age at diagnosis was available in 84 % of the patients. Patients who suffer from the severe form of the disease are typically diagnosed shortly after the first year of life, whereas non-severe patients were most likely to be diagnosed after the age of 4 years (with a median of 7 years of age).
Data is available on the treatment modalities of 94 % of the patients included in the database: prophylaxis is applied in 71 % of the patients with severe haemophilia, whereas 29 % receive on demand treatment. The number of severe haemophiliacs on prophylaxis was higher among children (91 %) than among adults (62 %). Regarding the type of product, 71 % of all patients with severe haemophilia have a recombinant product. In 72 % of all severe HA patients, a recombinant product is used, whereas this percentage is only 62.5 % in severe HB patients. Data on the viral status is available of 71.4 % of the patients with severe haemophilia. Overall, 13 % of all patients with severe HA are infected with HIV and 37 % are HCV-positive; a co-infection with both, HIV and HCV, has been confirmed in 10.7 %. Severe HB patients were HIV-positive in 4 %, HCV-positive in 37.5 % and in 4 % a co-infection with HIV and HCV was present. Currently, 3.6 % of all HA and 7.8 % of the severe HA patients have an inhibitor – 52 % of all HA patients with inhibitor and 57 % of the severe HA patients with inhibitor have high-titer inhibitor (> 5.0 Bethesda Units (BU)/ml). Presently 0.8 % of all our HB patients and 4 % of severe HB patients have a low-titer inhibitor (< 5.0 BU/ml).
Conclusion
The Austrian Haemophilia Registry enables us to obtain epidemiological data on haemophilia in Austria. The Registry also supports us in the effective planning of our scientific projects concerning bleeding disorders.
References
1. Reitter, S. et al, Wien Klin Wochenschr 2009; 121: 196–201