Glanzmann thrombasthenia successfully operated for nasal deformation with recombinant factor VIIA.

INDIAN PEDIATRICS 1094 VOLUME 43__DECEMBER 17, 2006 An 8-year-old boy diagnosed as Glanzmann thrombasthenia (GT) by other center was referred to our department in March 2005. He has been followed with GT for 4 years. He complained difficulty in breathing and recurrent upper respiratory tract infections. We observed a big perforation at nasal septum and granulation tissue related with forgotten nasal tampon on nasal examination. Results of complete blood count were as follows: hemoglobin 8.9 g/dL, mean corpuscular volume (MCV): 64 fl, white blood cell count 4800/mm3, and platelet count 321000/mm3. He was hospitalized for cleaning of the granulation tissue. In our patient alloimmunisation had developed due to frequently platelet transfusion to stop resistant nasal and gingival bleedings before the admission. The patient was operated with local anesthesia. Forgotten tampon was removed and granulation tissue was resected. Regeneration of septum nasi was performed. Bolus injection of Recombinant activated factor VIIa (rFVIIa) 90 μg/kg was given immediately before operation and three times with 2 hours intervals after the surgery. The patient was discharged one day after without any complication.