Bleeding pattern and consumption of factor VIII concentrate in
adult patients with haemophilia A without inhibitors in the
Czech Republic between 2013 and 2021 (Czech National
Haemophilia Programme registry data)

J 2023

Bleeding pattern and consumption of factor VIII concentrate in adult patients with haemophilia A without inhibitors in the Czech Republic between 2013 and 2021 (Czech National Haemophilia Programme registry data)

ROMANOVÁ, Gabriela, Petr SMEJKAL, Petra OVESNÁ, Eva DRBOHLAVOVA, Petr DULICEK et. al.

Basic information

Original name

Bleeding pattern and consumption of factor VIII concentrate in adult patients with haemophilia A without inhibitors in the Czech Republic between 2013 and 2021 (Czech National Haemophilia Programme registry data)

Authors

ROMANOVÁ, Gabriela (203 Czech Republic, belonging to the institution), Petr SMEJKAL (203 Czech Republic, belonging to the institution), Petra OVESNÁ (203 Czech Republic), Eva DRBOHLAVOVA (203 Czech Republic), Petr DULICEK (203 Czech Republic), Zdenka HAJSMANOVA (203 Czech Republic), Antonin HLUSI (203 Czech Republic), Radka HRDLICKOVA (203 Czech Republic), Jana ULLRYCHOVA (203 Czech Republic), Ivan VONKE (203 Czech Republic), Jan BLATNÝ (203 Czech Republic, belonging to the institution), Zuzana CERMAKOVA (203 Czech Republic), Ester ZAPOTOCKA (203 Czech Republic) and Miroslav PENKA (203 Czech Republic, belonging to the institution)

Edition

Annals of hematology, New York, Springer Verlag, 2023, 0939-5555

Other information

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30205 Hematology

Country of publisher

United States of America

Confidentiality degree

není předmětem státního či obchodního tajemství

References:

URL

Impact factor

Impact factor: 3.500 in 2022

RIV identification code

RIV/00216224:14110/23:00132991

Organization unit

Faculty of Medicine

DOI

http://dx.doi.org/10.1007/s00277-023-05453-6

UT WoS

001071705000001

Keywords in English

Haemophilia; Bleeding; Prophylaxis; On-demand; Consumption of factor VIII/IX concentrate; CNHP (the Czech National Haemophilia Programme); EHL (extended half-life)

Abstract

V originále

The manuscript provides an overview of treatment and its changes in adult patients with haemophilia A without inhibitors in the Czech Republic between 2013 and 2021 using data from the registry of the Czech National Haemophilia Programme (CNHP). Over a 9-year period, we focused on the reduction in the annual bleeding rate (ABR), joint bleeding rate (AJBR) and factor VIII consumption when patients with severe haemophilia A switched from on-demand treatment to prophylaxis. The ABR and AJBR include both patient-reported home treatment and treated hospitalisation episodes. All adult patients with severe haemophilia A were categorised into three groups according to the therapeutic regimen. The first group was patients on prophylaxis during the follow-up period, the second group consisted of patients on on-demand treatment, and the third group was patients who received both treatment regimens during follow-up. With an increase in the proportion of patients with severe haemophilia A on prophylaxis from 37 to 74% between 2013 and 2021, the ABR for all patients with severe haemophilia A decreased approximately 6.9-fold, and the AJBR decreased 8.7-fold. Expectedly, the factor consumption increased by approximately 68.5%. In the group of patients with severe haemophilia A who had switched from an on-demand to a prophylactic regimen, the total number of bleeding events decreased 3.5-fold, and the number of joint bleeding episodes decreased 3.9-fold. Factor VIII consumption increased by 78.4%. Our study supports a previously reported positive effect of prophylaxis on bleeding control. We believe that the substantial improvement in ABR justifies the increased treatment costs.

Citovat

ROMANOVÁ, Gabriela, Petr SMEJKAL, Petra OVESNÁ, Eva DRBOHLAVOVA, Petr DULICEK, Zdenka HAJSMANOVA, Antonin HLUSI, Radka HRDLICKOVA, Jana ULLRYCHOVA, Ivan VONKE, Jan BLATNÝ, Zuzana CERMAKOVA, Ester ZAPOTOCKA and Miroslav PENKA. Bleeding pattern and consumption of factor VIII concentrate in adult patients with haemophilia A without inhibitors in the Czech Republic between 2013 and 2021 (Czech National Haemophilia Programme registry data). Annals of hematology. New York: Springer Verlag, 2023, vol. 102, No 11, p. 3261-3270. ISSN 0939-5555. Available from: https://dx.doi.org/10.1007/s00277-023-05453-6.

@article{2360048,
   author = {Romanová, Gabriela and Smejkal, Petr and Ovesná, Petra and Drbohlavova, Eva and Dulicek, Petr and Hajsmanova, Zdenka and Hlusi, Antonin and Hrdlickova, Radka and Ullrychova, Jana and Vonke, Ivan and Blatný, Jan and Cermakova, Zuzana and Zapotocka, Ester and Penka, Miroslav},
   article_location = {New York},
   article_number = {11},
   doi = {http://dx.doi.org/10.1007/s00277-023-05453-6},
   keywords = {Haemophilia; Bleeding; Prophylaxis; On-demand; Consumption of factor VIII/IX concentrate; CNHP (the Czech National Haemophilia Programme); EHL (extended half-life)},
   language = {eng},
   issn = {0939-5555},
   journal = {Annals of hematology},
   title = {Bleeding pattern and consumption of factor VIII concentrate in adult patients with haemophilia A without inhibitors in the Czech Republic between 2013 and 2021 (Czech National Haemophilia Programme registry data)},
   url = {https://link.springer.com/article/10.1007/s00277-023-05453-6},
   volume = {102},
   year = {2023}
}

TY  - JOUR
ID  - 2360048
AU  - Romanová, Gabriela - Smejkal, Petr - Ovesná, Petra - Drbohlavova, Eva - Dulicek, Petr - Hajsmanova, Zdenka - Hlusi, Antonin - Hrdlickova, Radka - Ullrychova, Jana - Vonke, Ivan - Blatný, Jan - Cermakova, Zuzana - Zapotocka, Ester - Penka, Miroslav
PY  - 2023
TI  - Bleeding pattern and consumption of factor VIII concentrate in adult patients with haemophilia A without inhibitors in the Czech Republic between 2013 and 2021 (Czech National Haemophilia Programme registry data)
JF  - Annals of hematology
VL  - 102
IS  - 11
SP  - 3261-3270
EP  - 3261-3270
PB  - Springer Verlag
SN  - 09395555
KW  - Haemophilia
KW  - Bleeding
KW  - Prophylaxis
KW  - On-demand
KW  - Consumption of factor VIII/IX concentrate
KW  - CNHP (the Czech National Haemophilia Programme)
KW  - EHL (extended half-life)
UR  - https://link.springer.com/article/10.1007/s00277-023-05453-6
N2  - The manuscript provides an overview of treatment and its changes in adult patients with haemophilia A without inhibitors in the Czech Republic between 2013 and 2021 using data from the registry of the Czech National Haemophilia Programme (CNHP). Over a 9-year period, we focused on the reduction in the annual bleeding rate (ABR), joint bleeding rate (AJBR) and factor VIII consumption when patients with severe haemophilia A switched from on-demand treatment to prophylaxis. The ABR and AJBR include both patient-reported home treatment and treated hospitalisation episodes. All adult patients with severe haemophilia A were categorised into three groups according to the therapeutic regimen. The first group was patients on prophylaxis during the follow-up period, the second group consisted of patients on on-demand treatment, and the third group was patients who received both treatment regimens during follow-up. With an increase in the proportion of patients with severe haemophilia A on prophylaxis from 37 to 74% between 2013 and 2021, the ABR for all patients with severe haemophilia A decreased approximately 6.9-fold, and the AJBR decreased 8.7-fold. Expectedly, the factor consumption increased by approximately 68.5%. In the group of patients with severe haemophilia A who had switched from an on-demand to a prophylactic regimen, the total number of bleeding events decreased 3.5-fold, and the number of joint bleeding episodes decreased 3.9-fold. Factor VIII consumption increased by 78.4%. Our study supports a previously reported positive effect of prophylaxis on bleeding control. We believe that the substantial improvement in ABR justifies the increased treatment costs.
ER  -

ROMANOVÁ, Gabriela, Petr SMEJKAL, Petra OVESNÁ, Eva DRBOHLAVOVA, Petr DULICEK, Zdenka HAJSMANOVA, Antonin HLUSI, Radka HRDLICKOVA, Jana ULLRYCHOVA, Ivan VONKE, Jan BLATNÝ, Zuzana CERMAKOVA, Ester ZAPOTOCKA and Miroslav PENKA. Bleeding pattern and consumption of factor VIII concentrate in adult patients with haemophilia A without inhibitors in the Czech Republic between 2013 and 2021 (Czech National Haemophilia Programme registry data). \textit{Annals of hematology}. New York: Springer Verlag, 2023, vol.~102, No~11, p.~3261-3270. ISSN~0939-5555. Available from: https://dx.doi.org/10.1007/s00277-023-05453-6.

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