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

J 2015

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

BLATNY, Jan; Vladimir KOMRSKA; Bohumir BLAZEK; Miroslav PENKA; Petra OVESNÁ et al.

Základní údaje

Originální název

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

Autoři

BLATNY, Jan; Vladimir KOMRSKA; Bohumir BLAZEK; Miroslav PENKA a Petra OVESNÁ

Vydání

Blood Coagulation and Fibrinolysis, Philadelphia, Lippincott Williams and Wilkins, 2015, 0957-5235

Další údaje

Jazyk

angličtina

Typ výsledku

Článek v odborném periodiku

Obor

30200 3.2 Clinical medicine

Stát vydavatele

Spojené státy

Utajení

není předmětem státního či obchodního tajemství

Impakt faktor

Impact factor: 1.242

Označené pro přenos do RIV

Ano

Kód RIV

RIV/00216224:14110/15:00085183

Organizační jednotka

Lékařská fakulta

Klíčová slova anglicky

bleeding; factor; haemophilia; inhibitors; recombinant

Štítky

EL OK

Příznaky

Mezinárodní význam, Recenzováno

Změněno: 10. 12. 2015 15:23, Soňa Böhmová

Anotace

V originále

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.

Návaznosti

EE2.4.12.0048, projekt VaV

Název: Vzdělávací síť hemofilických center

Citovat

BLATNY, Jan; Vladimir KOMRSKA; Bohumir BLAZEK; Miroslav PENKA a 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, roč. 26, č. 6, s. 673-678. ISSN 0957-5235. Dostupné z: https://doi.org/10.1097/MBC.0000000000000298.

@article{1320677,
   author = {Blatny, Jan and Komrska, Vladimir and Blazek, Bohumir and Penka, Miroslav and Ovesná, Petra},
   article_location = {Philadelphia},
   article_number = {6},
   doi = {https://doi.org/10.1097/MBC.0000000000000298},
   keywords = {bleeding; factor; haemophilia; inhibitors; recombinant},
   language = {eng},
   issn = {0957-5235},
   journal = {Blood Coagulation and Fibrinolysis},
   title = {Inhibitors incidence rate in Czech previously untreated patients with haemophilia A has not increased since introduction of recombinant factor VIII treatment in 2003},
   volume = {26},
   year = {2015}
}

TY  - JOUR
ID  - 1320677
AU  - Blatny, Jan - Komrska, Vladimir - Blazek, Bohumir - Penka, Miroslav - Ovesná, Petra
PY  - 2015
TI  - Inhibitors incidence rate in Czech previously untreated patients with haemophilia A has not increased since introduction of recombinant factor VIII treatment in 2003
JF  - Blood Coagulation and Fibrinolysis
VL  - 26
IS  - 6
SP  - 673-678
EP  - 673-678
PB  - Lippincott Williams and Wilkins
SN  - 09575235
KW  - bleeding
KW  - factor
KW  - haemophilia
KW  - inhibitors
KW  - recombinant
N2  - 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.
ER  -

BLATNY, Jan; Vladimir KOMRSKA; Bohumir BLAZEK; Miroslav PENKA a 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. \textit{Blood Coagulation and Fibrinolysis}. Philadelphia: Lippincott Williams and Wilkins, 2015, roč.~26, č.~6, s.~673-678. ISSN~0957-5235. Dostupné z: https://doi.org/10.1097/MBC.0000000000000298.

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