The use of recombinant FVIIa in a patient with Glanzmann thrombasthenia with uncontrolled bleeding after tonsillectomy.
Glanzmann's thrombasthenia is an inherited platelet function disorder caused by quantitative or qualitative defects of the platelet membrane glycoprotein IIb/IIIa complex. Activated recombinant factor VII (rFVIIa) has recently been used in the treatment of patients with Glanzmann's thrombasthenia. We report herein a 16-year-old boy with Glanzmann's thrombasthenia who did not respond to conservative treatment for excessive bleeding and hyperfibrinolysis after tonsillectomy and who was successfully treated with rFVIIa. We suggest that rFVIIa at repeated doses of 80-100 microg/kg may be used effectively in patients with Glanzmann's thrombasthenia having excessive bleeding associated with hyperfibrinolysis after tonsillectomy in addition to tranexamic acid treatment.
Erduran
Erol
E
Pediatric Hematology Division, Turkey, Karadeniz Technical University, 61080 Trabzon, Turkey.
Aksoy
Ayşe
A
Zaman
Dilek
D
D000293:Adolescent; D016063:Blood Loss, Surgical / Q000517:prevention & control*; D015942:Factor VIIa / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D011994:Recombinant Proteins / Q000627:therapeutic use; D013915:Thrombasthenia / Q000503:physiopathology* / Q000628:therapy*; D014068:Tonsillectomy / Q000009:adverse effects*
D011994:Recombinant Proteins; C103587:recombinant FVIIa; D015942:Factor VIIa