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An illustrative case and a review on the dosing of recombinant factor VIIa in congenital factor XI deficiency
Author(s) -
SCHULMAN S.,
NÉMETH G.
Publication year - 2006
Publication title -
haemophilia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.213
H-Index - 92
eISSN - 1365-2516
pISSN - 1351-8216
DOI - 10.1111/j.1365-2516.2006.01276.x
Subject(s) - medicine , recombinant factor viia , dosing , factor viia , bolus (digestion) , haemophilia , recombinant dna , coagulopathy , factor xi , factor ix , haemophilia a , surgery , pediatrics , anesthesia , coagulation , tissue factor , biochemistry , chemistry , gene
Summary. Recombinant activated factor VII (rFVIIa) has been used in a very limited number of patients with severe factor XI (FXI) deficiency. The dose and duration of treatment has varied greatly between these case reports. In a few of these cases there was also evidence of thrombotic complications. We present here a report on one additional patient with congenital FXI deficiency. For two major orthopaedic procedures in this patient we used rFVIIa as a single bolus dose followed by continuous infusion at a low rate. The data from these treatment episodes, together with those from a review of the published cases, lend support to the concept of using much lower doses than in haemophilia with inhibitors. A bolus dose of 20 μ g kg −1 and thereafter maintenance of the FVII activity at approximately 3 IU mL −1 appears effective and safe.