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Thrombin generation in vitro in the presence of by-passing agents in siblings with severe haemophilia A.

Klintman, Jenny LU ; Berntorp, Erik LU and Astermark, Jan LU (2010) In Haemophilia 16. p.210-215
Abstract
Summary. Previous data have shown an inter-individual difference in the thrombin generating capacity in vitro as well as phenotypic bleeding pattern among patients with severe haemophilia A (FVIII:C activity below 1%). The reason for this is not known. In addition, there are no reports on how thrombin generation may correlate between siblings. In this study, we evaluated and compared thrombin generation in vitro using plasma samples in the presence of by-passing agents (FEIBA((R)) and NovoSeven((R))) in 21 unrelated brother pairs with and without inhibitors enrolled in the Malmö International Brother Study (MIBS). Mean maximum thrombin formation in patients with a current inhibitor titer was 182.0 +/- 52.8 mmol mL(-1) (FEIBA((R))) and... (More)
Summary. Previous data have shown an inter-individual difference in the thrombin generating capacity in vitro as well as phenotypic bleeding pattern among patients with severe haemophilia A (FVIII:C activity below 1%). The reason for this is not known. In addition, there are no reports on how thrombin generation may correlate between siblings. In this study, we evaluated and compared thrombin generation in vitro using plasma samples in the presence of by-passing agents (FEIBA((R)) and NovoSeven((R))) in 21 unrelated brother pairs with and without inhibitors enrolled in the Malmö International Brother Study (MIBS). Mean maximum thrombin formation in patients with a current inhibitor titer was 182.0 +/- 52.8 mmol mL(-1) (FEIBA((R))) and 130.7 +/- 54.9 mmol mL(-1) (rFVIIa), respectively, and somewhat higher in those without inhibitors, 222.7 +/-85.5 mmol mL(-1) (FEIBA((R))) and 142.8 +/-53.6mmol mL(-1) (rFVIIa) (P = 0.16 and 0.29). The variance regarding the maximum thrombin production within a family was significantly lower compared with the thrombin production between families (P < 0.001 for both FEIBA((R)) and NovoSeven((R))). Our data indicate that genetically determined factors, other than the FVIII:C activity seems to influence the phenotypic variation in thrombin formation in the presence of by-passing agents. The nature of these determinants remains to be identified. (Less)
Please use this url to cite or link to this publication:
author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Haemophilia
volume
16
pages
210 - 215
publisher
Federation of European Neuroscience Societies and Blackwell Publishing Ltd
external identifiers
  • wos:000273179800032
  • pmid:19878339
  • scopus:73949097582
ISSN
1351-8216
DOI
10.1111/j.1365-2516.2009.02132.x
language
English
LU publication?
yes
id
033504bb-c83f-47b1-92ae-bcf0dc71acc6 (old id 1512440)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/19878339?dopt=Abstract
date added to LUP
2009-12-01 10:57:05
date last changed
2018-05-29 12:18:50
@article{033504bb-c83f-47b1-92ae-bcf0dc71acc6,
  abstract     = {Summary. Previous data have shown an inter-individual difference in the thrombin generating capacity in vitro as well as phenotypic bleeding pattern among patients with severe haemophilia A (FVIII:C activity below 1%). The reason for this is not known. In addition, there are no reports on how thrombin generation may correlate between siblings. In this study, we evaluated and compared thrombin generation in vitro using plasma samples in the presence of by-passing agents (FEIBA((R)) and NovoSeven((R))) in 21 unrelated brother pairs with and without inhibitors enrolled in the Malmö International Brother Study (MIBS). Mean maximum thrombin formation in patients with a current inhibitor titer was 182.0 +/- 52.8 mmol mL(-1) (FEIBA((R))) and 130.7 +/- 54.9 mmol mL(-1) (rFVIIa), respectively, and somewhat higher in those without inhibitors, 222.7 +/-85.5 mmol mL(-1) (FEIBA((R))) and 142.8 +/-53.6mmol mL(-1) (rFVIIa) (P = 0.16 and 0.29). The variance regarding the maximum thrombin production within a family was significantly lower compared with the thrombin production between families (P &lt; 0.001 for both FEIBA((R)) and NovoSeven((R))). Our data indicate that genetically determined factors, other than the FVIII:C activity seems to influence the phenotypic variation in thrombin formation in the presence of by-passing agents. The nature of these determinants remains to be identified.},
  author       = {Klintman, Jenny and Berntorp, Erik and Astermark, Jan},
  issn         = {1351-8216},
  language     = {eng},
  pages        = {210--215},
  publisher    = {Federation of European Neuroscience Societies and Blackwell Publishing Ltd},
  series       = {Haemophilia},
  title        = {Thrombin generation in vitro in the presence of by-passing agents in siblings with severe haemophilia A.},
  url          = {http://dx.doi.org/10.1111/j.1365-2516.2009.02132.x},
  volume       = {16},
  year         = {2010},
}