Control of severe bleeding episode in case of Glanzmann's thrombasthenia refractory to platelet transfusion therapy by administering recombinant factor VIIa.
Glanzmann's thrombasthenia is an autosomal recessive inherited platelet function defect. Though, quantitatively normal, the aggregation ability of platelets is reduced leading to bleeding episodes requiring transfusion of platelet concentrates. We describe a case of 13-year-old girl who had recurrent episodes of epistaxis since birth and was managed with multiple platelet concentrate transfusions and recently admitted with severe epistaxis refractory to platelet transfusion. At this stage administration of recombinant activated factor VII (fVIIa) was considered, which was initially given at 90 microg/kg dose with little control of bleeding but subsequent second dose of 120 microg/kg was administered with excellent response and immediate control of bleeding.
Javed
Asim
A
Combined Military Hospital, Rawalpindi, Pakistan.
Ayyub
Muhammad
M
Abrar
S
S
Mansoor
M
M
Khan
Badshah
B
Hussain
T
T
D000293:Adolescent; D004844:Epistaxis / Q000517:prevention & control*; D015942:Factor VIIa / Q000008:administration & dosage / Q000627:therapeutic use*; D005260:Female; D006470:Hemorrhage / Q000517:prevention & control*; D006801:Humans; D010974:Platelet Aggregation; D017713:Platelet Transfusion; D011994:Recombinant Proteins / Q000008:administration & dosage / Q000627:therapeutic use; D013915:Thrombasthenia / Q000150:complications* / Q000628:therapy; D017211:Treatment Failure
D011994:Recombinant Proteins; C103587:recombinant FVIIa; D015942:Factor VIIa