Document Detail

The plasma concentration of activated protein C appears normal in patients with haemophilia.
MedLine Citation:
PMID:  19175419     Owner:  NLM     Status:  MEDLINE    
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 microg 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.
S Schulman; A Eelde; M Blombäck; J Stenflo
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Publication Detail:
Type:  Journal Article     Date:  2009-01-20
Journal Detail:
Title:  Haemophilia : the official journal of the World Federation of Hemophilia     Volume:  15     ISSN:  1365-2516     ISO Abbreviation:  Haemophilia     Publication Date:  2009 Mar 
Date Detail:
Created Date:  2009-04-01     Completed Date:  2009-12-14     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9442916     Medline TA:  Haemophilia     Country:  England    
Other Details:
Languages:  eng     Pagination:  566-70     Citation Subset:  IM    
Department of Hematology, Karolinska University Hospital, Stockholm, Sweden.
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MeSH Terms
Analysis of Variance
Biological Markers / blood
Enzyme Activation
Hemophilia A / blood*,  genetics
Hemophilia B / blood*,  genetics
Middle Aged
Predictive Value of Tests
Protein C / genetics,  metabolism*
Thrombin / genetics,  metabolism*
Reg. No./Substance:
0/Biological Markers; 0/Protein C; EC

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