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Inhibitors incidence rate in Czech previously untreated patients with haemophilia A has not increased since introduction of recombinant factor VIII treatment in 2003

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BLATNY Jan KOMRSKA Vladimir BLAZEK Bohumir PENKA Miroslav OVESNÁ Petra

Rok publikování 2015
Druh Článek v odborném periodiku
Časopis / Zdroj Blood Coagulation and Fibrinolysis
Fakulta / Pracoviště MU

Lékařská fakulta

Citace
Doi http://dx.doi.org/10.1097/MBC.0000000000000298
Obor Onkologie a hematologie
Klíčová slova bleeding; factor; haemophilia; inhibitors; recombinant
Popis 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.
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