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The plasma concentration of activated protein C appears normal in patients with haemophilia

Schulman, S.; Eelde, A.; Blomback, M. and Stenflo, Johan LU (2009) In Haemophilia 15(2). p.566-570
Abstract
Increased concentration of activated protein C (APC) has been observed in patients with thromboembolic disorders, but whether the level of APC in patients with bleeding disorders is decreased remains unknown. Seventy patients with haemophilia A or B with mild, moderate or severe form were studied. Detailed information on bleeding, arthropathy and factor consumption was collected during a 10-year period. The clinical severity of the disease was expressed as the Hemophilia Severity Score (HSS). Plasma concentration of APC was measured as APC in complex with protein C inhibitor. The median concentration of APC-PCI complex in patients with haemophilia was 0.14 mu g L-1 and it did not differ between the types and forms. In 16 patients with... (More)
Increased concentration of activated protein C (APC) has been observed in patients with thromboembolic disorders, but whether the level of APC in patients with bleeding disorders is decreased remains unknown. Seventy patients with haemophilia A or B with mild, moderate or severe form were studied. Detailed information on bleeding, arthropathy and factor consumption was collected during a 10-year period. The clinical severity of the disease was expressed as the Hemophilia Severity Score (HSS). Plasma concentration of APC was measured as APC in complex with protein C inhibitor. The median concentration of APC-PCI complex in patients with haemophilia was 0.14 mu g L-1 and it did not differ between the types and forms. In 16 patients with severe haemophilia A and the inversion mutation in intron 22, there was no correlation between clinical severity and the concentration of APC-PCI complex. Patients with haemophilia appear to generate normal concentrations of APC during basal conditions. APC does not seem to be an important modulator of the phenotypic expression of haemophilia. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
haemophilia, activated protein C, bleeding, phenotype
in
Haemophilia
volume
15
issue
2
pages
566 - 570
publisher
Federation of European Neuroscience Societies and Blackwell Publishing Ltd
external identifiers
  • wos:000264546900019
  • scopus:63349111957
ISSN
1351-8216
DOI
10.1111/j.1365-2516.2008.01957.x
language
English
LU publication?
yes
id
84345b25-590d-483a-8360-2b9bc82a1e45 (old id 1401064)
date added to LUP
2009-06-15 12:39:56
date last changed
2017-01-01 04:51:38
@article{84345b25-590d-483a-8360-2b9bc82a1e45,
  abstract     = {Increased concentration of activated protein C (APC) has been observed in patients with thromboembolic disorders, but whether the level of APC in patients with bleeding disorders is decreased remains unknown. Seventy patients with haemophilia A or B with mild, moderate or severe form were studied. Detailed information on bleeding, arthropathy and factor consumption was collected during a 10-year period. The clinical severity of the disease was expressed as the Hemophilia Severity Score (HSS). Plasma concentration of APC was measured as APC in complex with protein C inhibitor. The median concentration of APC-PCI complex in patients with haemophilia was 0.14 mu g L-1 and it did not differ between the types and forms. In 16 patients with severe haemophilia A and the inversion mutation in intron 22, there was no correlation between clinical severity and the concentration of APC-PCI complex. Patients with haemophilia appear to generate normal concentrations of APC during basal conditions. APC does not seem to be an important modulator of the phenotypic expression of haemophilia.},
  author       = {Schulman, S. and Eelde, A. and Blomback, M. and Stenflo, Johan},
  issn         = {1351-8216},
  keyword      = {haemophilia,activated protein C,bleeding,phenotype},
  language     = {eng},
  number       = {2},
  pages        = {566--570},
  publisher    = {Federation of European Neuroscience Societies and Blackwell Publishing Ltd},
  series       = {Haemophilia},
  title        = {The plasma concentration of activated protein C appears normal in patients with haemophilia},
  url          = {http://dx.doi.org/10.1111/j.1365-2516.2008.01957.x},
  volume       = {15},
  year         = {2009},
}