BLATNY, Jan, Vladimir KOMRSKA, Bohumir BLAZEK, Miroslav PENKA and Petra OVESNÁ. Inhibitors incidence rate in Czech previously untreated patients with haemophilia A has not increased since introduction of recombinant factor VIII treatment in 2003. Blood Coagulation and Fibrinolysis. Philadelphia: Lippincott Williams and Wilkins, 2015, vol. 26, No 6, p. 673-678. ISSN 0957-5235. Available from: https://dx.doi.org/10.1097/MBC.0000000000000298.
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Basic information
Original name Inhibitors incidence rate in Czech previously untreated patients with haemophilia A has not increased since introduction of recombinant factor VIII treatment in 2003
Authors BLATNY, Jan (203 Czech Republic), Vladimir KOMRSKA (203 Czech Republic), Bohumir BLAZEK (203 Czech Republic), Miroslav PENKA (203 Czech Republic) and Petra OVESNÁ (203 Czech Republic, guarantor, belonging to the institution).
Edition Blood Coagulation and Fibrinolysis, Philadelphia, Lippincott Williams and Wilkins, 2015, 0957-5235.
Other information
Original language English
Type of outcome Article in a journal
Field of Study 30200 3.2 Clinical medicine
Country of publisher United States of America
Confidentiality degree is not subject to a state or trade secret
Impact factor Impact factor: 1.242
RIV identification code RIV/00216224:14110/15:00085183
Organization unit Faculty of Medicine
Doi http://dx.doi.org/10.1097/MBC.0000000000000298
UT WoS 000364400500013
Keywords in English bleeding; factor; haemophilia; inhibitors; recombinant
Tags EL OK
Tags International impact, Reviewed
Changed by Changed by: Soňa Böhmová, učo 232884. Changed: 10/12/2015 15:23.
Abstract
Our objective was to assess the incidence of inhibitors development in Czech Republic since the introduction of recombinant factor VIII (rFVIII) and to look for the factors potentially influencing this parameter. It is to be expected that inhibitors risk may be increased after the introduction of recombinant products. Data of Czech National Haemophilia Programme registry entered from 2003 till 2013 were analysed. Both annual and absolute incidences of newly developed inhibitors in previously untreated patients (PUPs) were calculated. Bleeding and treatment data were also extracted, and association to the treatment regimen and development of inhibitors were analysed. Within the given period, we commenced 45 PUPs with haemophilia A on rFVIII and treated them for 137 treatment-years. Twenty-two of the PUPs had severe haemophilia A, being treated for 88 treatment-years. Treatment strategy for them was prophylaxis. Other PUPs were treated on demand. Median annual bleeding rate was 5 for boys with severe haemophilia, 3 for moderate haemophilia and 1 for mild form of the disease. No inhibitors developed in PUPs with moderate/mild haemophilia A. Annual inhibitor incidence rate in PUPs with severe haemophilia A treated with rFVIII was 56.8 per 1000 treatment-years. Absolute incidence was 22.7% (5/22). All inhibitors appeared within the first 50 exposure days. Comparing rFVIII-treated group with the control group treated under same/similar conditions with plasma-derived FVIII during the same follow-up period, we were not able to find significant difference in inhibitor development between these two groups. Our results support the finding that use of rFVIII is not a proven risk factor for inhibitor development in patients with haemophilia A.
Links
EE2.4.12.0048, research and development projectName: Vzdělávací síť hemofilických center
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