Efficacy of Recombinant Activated Factor VII in the Treatment of Bleeding in Hemophilia Children with Inhibitor: Ten Year Experience from 1997 to 2007
คำสำคัญ:
Hemophilia with inhibitor, Recombinant activated factor VIIบทคัดย่อ
Abstract :
We report the efficacy of recombinant activated factor VII (rFVIIa) in the treatment of 82
bleeding episodes among 10 severe hemophilia children (A9, B1) with a median inhibitor of 18.3 BU
(interquartile range 3.9-100.0 BU). The bleeding episodes included bleeding at various sites such as
hemarthrosis and hematoma at the muscle (n=70), dental procedures (n=5) and surgery (n=7). Local
measure combined with symptomatic treatment were additionally provided. Bleeding at various sites
were divided into 2 groups: group 1, 23 cases receiving rFVIIa <150 mcg/kg (median 100 interquartile
range 90-120) and group 2, 47 cases receiving rFVIIa >150 mcg/kg (median 200, interquartile range 180-
200). The repeated doses of rFVIIa were given at an interval of 2-3 h according to the clinical manifestation.
The results revealed that the effective responses of patients in group 1 were similar to those
of group 2 at 12 h (60.9% vs 63.8%), 18 h (70.9% vs 80.9%), 24 h (87.0% vs 91.5%) and 48 h (95.7% vs
95.7%). The median dose per bleed in group 1 was two doses which was significantly higher than
one dose of group 2 with a p value of 0.032. However, the median total amount per bleed in both groups
was similar at 184.6 and 20.43 mcg/kg. In addition, the dental procedures were effectively performed
by giving 1-2 doses of rFVIIa combined with fibrin glue and splint. Lastly, 5 major surgeries were
uneventfully performed by giving a 90-100 mcg/kg of rFVIIa at 2-4 h interval while 2 minor surgeries
required only 1-2 doses of rFVIIa. All patients survived and no adverse reaction was found. In conclusion,
rFVIIa has shown an effectiveness in the treatment of bleeding in hemophiliac children with
inhibitor. Since the prize of rFVIIa is very high, the appropriate use of rFVIIa is emphasized.
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