Document Detail

Potential role of the dynamic properties of whole blood coagulation in assessment of dosage requirements in haemophilia.
MedLine Citation:
PMID:  12828667     Owner:  NLM     Status:  MEDLINE    
In the clinical setting, patients suffering from haemophilia are classified according to the residual level of the deficient coagulation factor. Patients suffering from the severe form of haemophilia (critical factor level <0.01 IU mL-1) display some heterogeneity in their tendency to bleeding despite the uniform factor level. Utilizing a new thrombelastographic method in which coagulation is activated by very small amounts of tissue factor and where resulting data are processed with new software, we studied the whole blood coagulation profile in 11 patients with severe haemophilia A and 11 patients with moderate haemophilia. In both groups of patients, we found a considerable degree of heterogeneity in the coagulation signal. In moderate haemophilia with factor VIII (FVIII):C levels between 0.01 and 0.05 IU mL-1, variance was expected, whereas a quite substantial diversity had not been forecasted in patterns of the whole blood coagulation profiles in patients with the severe form of haemophilia A. Ex vivo substitution to patient's blood to reach various theoretical levels of recombinant factor VIII (rFVIII) revealed that the coagulation response to FVIII supplementation varied substantially. In some severely affected patients levels of FVIII:C close to 0.05 IU mL-1 was sufficient to normalize the coagulation profile, while others required a dose giving >0.50 IU mL-1 of FVIII to achieve a normal whole blood clotting profile. In conclusion, our study revealed that severe haemophilia A seems not to be a single entity, but rather several different clinical and biochemical phenotypes, and that the response to added FVIII varies amongst patients.
J Ingerslev; L H Poulsen; B Sørensen
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Haemophilia : the official journal of the World Federation of Hemophilia     Volume:  9     ISSN:  1351-8216     ISO Abbreviation:  Haemophilia     Publication Date:  2003 Jul 
Date Detail:
Created Date:  2003-06-27     Completed Date:  2003-10-07     Revised Date:  2009-10-21    
Medline Journal Info:
Nlm Unique ID:  9442916     Medline TA:  Haemophilia     Country:  England    
Other Details:
Languages:  eng     Pagination:  348-52     Citation Subset:  IM    
Centre for Haemophilia and Thrombosis, Department of Clinical Biochemistry, University Hospital Skejby, Aarhus N, Denmark.
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MeSH Terms
Blood Coagulation / drug effects*
Blood Coagulation Tests / methods
Dose-Response Relationship, Drug
Drug Administration Schedule
Factor VIII / administration & dosage*,  therapeutic use
Hemophilia A / blood,  drug therapy*
Recombinant Proteins / administration & dosage,  therapeutic use
Thrombelastography / methods
Reg. No./Substance:
0/Recombinant Proteins; 9001-27-8/Factor VIII

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