Abstract:
:Inherited factor VII (FVII) deficiency is a rare autosomal recessive disorder associated with a bleeding tendency. We describe three patients with congenital FVII deficiency who have been treated with activated recombinant factor VII (rVIIa). Two patients had novel mutations and were treated prophylactically with 1.2 mg rVIIa two to three times a week. Patients 1 and 2 had a severe bleeding tendency. The frequency and severity of bleeding decreased by treatment with rVIIa compared with similar treatment with plasma-derived FVII. The third patient with a moderate bleeding phenotype was treated on demand and showed no change in the frequency of bleeding upon treatment with rVIIa or plasma products. The beneficial effect of rVIIa cannot be explained by the rVIIa half-lives. Pharmacokinetical analysis showed rVIIa activity half-lives of 35, 50 and 54 min for patients 1, 2 and 3, respectively. In conclusion, prophylactic treatment of FVII deficient patients with rVIIa appears to be applicable, safe and successful, although the mechanism of action remains to be elucidated.
journal_name
Br J Haematoljournal_title
British journal of haematologyauthors
Mathijssen NC,Masereeuw R,Verbeek K,Lavergne JM,Costa JM,van Heerde WL,Nováková IRdoi
10.1111/j.1365-2141.2004.04942.xsubject
Has Abstractpub_date
2004-05-01 00:00:00pages
494-9issue
4eissn
0007-1048issn
1365-2141pii
BJH4942journal_volume
125pub_type
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