The Use of Recombinant Factor Viia in a Child With Glanzmann Thrombasthenia

Abstract

Glanzmann thrombasthenia is an autosomal recessive disorder of platelet aggregation that is characterized by a life-long bleeding tendency due to quantitative and qualitative abnormalities of the platelet membrane complex glycoprotein IIb/IIIa (Gp IIb/IIIa). Platelet transfusion is the standard treatment for severe bleeding and surgical support is necessary in these patients. However, repeated platelet transfusions can result in alloimmunization, which makes subsequent transfusions ineffective. Recombinant activated factor VIIa (rFVIIa) has recently been introduced as an alternative to platelet transfusion for treatment of bleeding episodes and to cover surgery in patients with hereditary platelet function defects. We report a 8-year-old child with Glanzmann thrombasthenia. The patient had been treated by nasal tampon placement because of epistaxis three years ago in another hospital. We detected perforation of nasal septum and deformation of nasal bone due to granulation tissue induced by forgotten nasal tampon. Forgotten tampon was removed and granulation tissue was resected. Bolus injections of rFVIIa (90 _g/kg) was given immediately before operation and three times with 2 hours intervals after the surgery. The patient was discharged from hospital without any bleeding complication or thrombocyte replacement.