-
Something wrong with this record ?
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)
G. Romanová, P. Smejkal, P. Ovesná, E. Drbohlavová, P. Dulíček, Z. Hajšmanová, A. Hluší, R. Hrdličková, J. Ullrychová, I. Vonke, J. Blatný, Z. Čermáková, E. Zápotocká, M. Penka
Language English Country Germany
Document type Journal Article
Grant support
FNBr
Ministerstvo Zdravotnictví Ceské Republiky
65269705
Ministerstvo Zdravotnictví Ceské Republiky
NLK
ProQuest Central
from 1997-03-01
Medline Complete (EBSCOhost)
from 2000-01-01 to 1 year ago
Nursing & Allied Health Database (ProQuest)
from 1997-03-01
Health & Medicine (ProQuest)
from 1997-03-01
Springer Nature OA/Free Journals
from 1955-03-01
- MeSH
- Adult MeSH
- Factor VIII * therapeutic use MeSH
- Hemophilia A * drug therapy MeSH
- Hemorrhage * chemically induced epidemiology MeSH
- Middle Aged MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Follow-Up Studies MeSH
- Registries * MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Male MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Geographicals
- Czech Republic MeSH
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.
2nd Medical Faculty Charles University Prague Czech Republic
Blood Centre University Hospital Ostrava Ostrava Czech Republic
Charles University Prague Prague Czech Republic
Clinical Haematology Hospital České Budějovice České Budějovice Czech Republic
Department of Clinical Haematology Regional Hospital Liberec Liberec Czech Republic
Department of Clinical Haematology University Hospital Brno Brno Czech Republic
Department of Laboratory Methods Faculty of Medicine Masaryk University Brno Czech Republic
Department of Paediatric Haematology and Biochemistry University Hospital Brno Brno Czech Republic
Department of Paediatric Haematology and Oncology University Hospital Motol Prague Czech Republic
Faculty of Medicine Masaryk University Brno Czech Republic
Institute of Biostatistics and Analyses Ltd Brno Czech Republic
References provided by Crossref.org
- 000
- 00000naa a2200000 a 4500
- 001
- bmc25004212
- 003
- CZ-PrNML
- 005
- 20250206110018.0
- 007
- ta
- 008
- 250121s2023 gw f 000 0|eng||
- 009
- AR
- 024 7_
- $a 10.1007/s00277-023-05453-6 $2 doi
- 035 __
- $a (PubMed)37740066
- 040 __
- $a ABA008 $b cze $d ABA008 $e AACR2
- 041 0_
- $a eng
- 044 __
- $a gw
- 100 1_
- $a Romanová, Gabriela $u Department of Internal Medicine, Haematology and, Oncology University Hospital Brno, Brno, Czech Republic. romanova.gabriela@fnbrno.cz $u Faculty of Medicine, Masaryk University, Brno, Czech Republic. romanova.gabriela@fnbrno.cz $1 https://orcid.org/0000000329481226
- 245 10
- $a 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) / $c G. Romanová, P. Smejkal, P. Ovesná, E. Drbohlavová, P. Dulíček, Z. Hajšmanová, A. Hluší, R. Hrdličková, J. Ullrychová, I. Vonke, J. Blatný, Z. Čermáková, E. Zápotocká, M. Penka
- 520 9_
- $a 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.
- 650 _2
- $a lidé $7 D006801
- 650 12
- $a hemofilie A $x farmakoterapie $7 D006467
- 650 12
- $a faktor VIII $x terapeutické užití $7 D005169
- 650 _2
- $a dospělí $7 D000328
- 650 12
- $a registrace $7 D012042
- 650 _2
- $a mužské pohlaví $7 D008297
- 650 12
- $a krvácení $x chemicky indukované $x epidemiologie $7 D006470
- 650 _2
- $a lidé středního věku $7 D008875
- 650 _2
- $a ženské pohlaví $7 D005260
- 650 _2
- $a mladý dospělý $7 D055815
- 650 _2
- $a mladiství $7 D000293
- 650 _2
- $a následné studie $7 D005500
- 651 _2
- $a Česká republika $x epidemiologie $7 D018153
- 655 _2
- $a časopisecké články $7 D016428
- 700 1_
- $a Smejkal, Petr $u Department of Clinical, Haematology University Hospital Brno, Brno, Czech Republic $u Department of Laboratory Methods Faculty of Medicine, Masaryk University, Brno, Czech Republic
- 700 1_
- $a Ovesná, Petra $u Institute of Biostatistics and Analyses, Ltd., Brno, Czech Republic
- 700 1_
- $a Drbohlavová, Eva $u Department of Clinical Haematology, Regional Hospital Liberec, Liberec, Czech Republic
- 700 1_
- $a Dulíček, Petr $u IVth Department of Internal Medicine-Haematology, University Hospital in Hradec Králové, Medical Faculty in Hradec Kralove, Hradec Králové, Czech Republic $u Charles University in Prague, Prague, Czech Republic
- 700 1_
- $a Hajšmanová, Zdeňka $u Department of Haematology, Institute of Clinical Biochemistry and, Haematology University Hospital Pilsen, Pilsen, Czech Republic
- 700 1_
- $a Hluší, Antonín $u Department of Haemato-Oncology, Faculty of Medicine and Dentistry, Palacky University and University Hospital Olomouc, Olomouc, Czech Republic
- 700 1_
- $a Hrdličková, Radka $u Blood Centre University Hospital Ostrava, Ostrava, Czech Republic
- 700 1_
- $a Ullrychová, Jana $u Department of Clinical Haematology Krajska Zdravotní a.S., Masaryk Hospital Ústí Nad Labem, Ústí Nad Labem, Czech Republic
- 700 1_
- $a Vonke, Ivan $u Clinical Haematology Hospital České Budějovice, České Budějovice, Czech Republic
- 700 1_
- $a Blatný, Jan $u Department of Paediatric Haematology and Biochemistry, University Hospital Brno, Brno, Czech Republic $u Masaryk University, Brno, Czech Republic
- 700 1_
- $a Čermáková, Zuzana $u Blood Centre University Hospital Ostrava, Ostrava, Czech Republic
- 700 1_
- $a Zápotocká, Ester $u Department of Paediatric Haematology and Oncology, University Hospital Motol, Prague, Czech Republic $u 2nd Medical Faculty, Charles University, Prague, Czech Republic
- 700 1_
- $a Penka, Miroslav $u Department of Internal Medicine, Haematology and, Oncology University Hospital Brno, Brno, Czech Republic $u Faculty of Medicine, Masaryk University, Brno, Czech Republic $u Department of Clinical, Haematology University Hospital Brno, Brno, Czech Republic
- 773 0_
- $w MED00000424 $t Annals of hematology $x 1432-0584 $g Roč. 102, č. 11 (2023), s. 3261-3270
- 856 41
- $u https://pubmed.ncbi.nlm.nih.gov/37740066 $y Pubmed
- 910 __
- $a ABA008 $b sig $c sign $y - $z 0
- 990 __
- $a 20250121 $b ABA008
- 991 __
- $a 20250206110013 $b ABA008
- 999 __
- $a ok $b bmc $g 2263758 $s 1240219
- BAS __
- $a 3
- BAS __
- $a PreBMC-MEDLINE
- BMC __
- $a 2023 $b 102 $c 11 $d 3261-3270 $e 20230923 $i 1432-0584 $m Annals of hematology $n Ann Hematol $x MED00000424
- GRA __
- $a FNBr $p Ministerstvo Zdravotnictví Ceské Republiky
- GRA __
- $a 65269705 $p Ministerstvo Zdravotnictví Ceské Republiky
- LZP __
- $a Pubmed-20250121
