Document Detail


Recombinant activated factor VII and tranexamic acid are haemostatically effective during major surgery in factor XI-deficient patients with inhibitor antibodies.
MedLine Citation:
PMID:  19718468     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
One-third of patients with severe factor XI (FXI) deficiency caused by homozygosity for null alleles develop inhibitor antibodies following exposure to plasma. Haemostasis during surgery is achievable in such patients by recombinant activated factor VII (rFVIIa) at doses used in haemophilia A patients with an inhibitor to FVIII. However, thrombosis has occurred in three of 12 such patients. In this study we discerned whether low-dose rFVIIa would secure haemostasis and cause no thrombosis in patients with severe FXI deficiency and an inhibitor during surgery. In vitro, a very low concentration of rFVIIa (0.24 microg/ml) induced thrombin generation in FXI-deficient plasma quite similarly to 1.9 microg/ml (a concentration that is achieved in patients with haemophilia A and inhibitor after infusion of 80 microg/kg). Based on this finding, a protocol was designed for four patients with severe FXI deficiency and an inhibitor or immunoglobulin A deficiency who underwent five major surgical procedures. This included administration of tranexamic acid from two hours before surgery until seven to 14 days after, and single infusion of low-dose rFVIIa. No excessive bleeding or thrombosis were observed. In conclusion, a single low dose of rFVIIa and tranexamic acid secure normal haemostasis in patients with severe FXI deficiency who can not receive blood products.
Authors:
Tami Livnat; Ilia Tamarin; Yoram Mor; Harry Winckler; Zeev Horowitz; Yoseph Korianski; Barak Bar-Zakay; Uri Seligsohn; Ophira Salomon
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Thrombosis and haemostasis     Volume:  102     ISSN:  0340-6245     ISO Abbreviation:  Thromb. Haemost.     Publication Date:  2009 Sep 
Date Detail:
Created Date:  2009-08-31     Completed Date:  2009-11-25     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7608063     Medline TA:  Thromb Haemost     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  487-92     Citation Subset:  IM    
Affiliation:
The Amalia Biron Research Institute of Thrombosis and Hemostasis, Sheba Medical Center, Tel Hashomer and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
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MeSH Terms
Descriptor/Qualifier:
Alleles
Blood Loss, Surgical / prevention & control*
Factor VIIa / metabolism*
Factor XI / antagonists & inhibitors*,  genetics*
Factor XI Deficiency / diagnosis,  genetics*
Female
Hemophilia A / genetics
Hemostasis
Homozygote
Humans
Male
Middle Aged
Thrombin / chemistry
Thrombosis
Time Factors
Tranexamic Acid / metabolism*
Chemical
Reg. No./Substance:
1197-18-8/Tranexamic Acid; 9013-55-2/Factor XI; EC 3.4.21.21/Factor VIIa; EC 3.4.21.5/Thrombin

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