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Blood, Vol. 94 No. 11 (December 1), 1999:
pp. 3951-3953
Recombinant Factor VIIa Is Effective for Bleeding and Surgery in
Patients With Glanzmann Thrombasthenia
Man-Chiu Poon,
Christine Demers,
François Jobin, and
John
W.Y. Wu
From the Departments of Medicine, Pediatrics, and Oncology,
University of Calgary and Foothills Hospital, Calgary, Alberta, Canada;
Southern Alberta Hemophilia Clinic, Alberta Children's Hospital,
Calgary, Alberta, Canada; Canadian Blood Services, Calgary
Centre, Calgary, Alberta, Canada; and Department d'hematologie,
Hôpital du Saint-Sacrement, Québec City, Québec,
Canada.
Recombinant activated factor VII (rFVIIa) was found to be effective
and safe in treating 24 bleeding episodes and to prevent bleeding
during one bilateral herniorrhaphy in four children with Glanzmann
thrombasthenia. One of the patients had alloantibodies to platelet
membrane glycoprotein (GP) IIb/IIIa and was refractory to platelet
transfusion. rFVIIa was administered at 89 to 116 µg/kg per injection
every 2 hours, in association with antifibrinolytic drugs. Bleeding
stopped in all cases, but platelet transfusion was required in one. Two
bleeding episodes recurred 36 and 63 hours after discontinuation of
rFVIIa, but were successfully treated with additional doses. No adverse
effects of rFVIIa were observed. Although the number of patients is
small, our study suggests that rFVIIa may be an alternative to platelet
transfusions in patients with a severe congenital thrombocytopathy.
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