title: Surgical resection of a sphenoid wing meningioma in a patient with Glanzmann thrombasthenia.
abstract: Glanzmann thrombasthenia (GT) is a rare autosomal recessive disorder characterized by a deficiency or functional defect of platelet glycoprotein (GP) IIb/IIIa. Physiologically, this platelet receptor mediates aggregation of activated platelets by binding the adhesive proteins, fibrinogen, von Willebrand factor (VWF) and fibronectin. This facilitates attachment and aggregation of platelets at sites of vascular injury. We reported the management of a pterional meningioma resection in a patient with Glanzmann thrombasthenia, with recombinant factor VIIa (rFVIIa - NovoSeven) as haemostatic agent. A 48-year-old woman suffering from Glanzmann thrombasthenia was scheduled for spheno-orbital meningioma en plaque surgery. Because of repeated platelet transfusions, this patient developed isoantibodies against missing GPIIbIIIa and alloantibodies against Human Leukocyte Antigen (HLA) leading to refractoriness to platelet transfusions. We observed that Novoseven offered sufficient haemostasis conditions. Therefore, we noticed a deep vein thrombosis. This imposed us to use low weight molecular heparin despite recent surgery.
authors.affiliation: Département d'Anesthésie-Réanimation, Université de Liège, Belgique. damien.wertz@chu.ulg.ac.be
authors.forename: D
authors.initials: D
authors.lastname: Wertz
authors.forename: P
authors.initials: P
authors.lastname: Boveroux
authors.forename: P
authors.initials: P
authors.lastname: Péters
authors.forename: J
authors.initials: J
authors.lastname: Lenelle
authors.forename: C
authors.initials: C
authors.lastname: Franssen
mesh_terms: D000925:Anticoagulants; D015942:Factor VIIa / Q000627:therapeutic use; D005260:Female; D006490:Hemostatics / Q000627:therapeutic use; D006495:Heparin, Low-Molecular-Weight / Q000627:therapeutic use; D006801:Humans; D008577:Meningeal Neoplasms / Q000150:complications / Q000601:surgery*; D008579:Meningioma / Q000150:complications / Q000601:surgery*; D008875:Middle Aged; D011994:Recombinant Proteins / Q000627:therapeutic use; D013915:Thrombasthenia / Q000150:complications*; D016896:Treatment Outcome; D020246:Venous Thrombosis / Q000150:complications
chemical_list: D000925:Anticoagulants; D006490:Hemostatics; D006495:Heparin, Low-Molecular-Weight; D011994:Recombinant Proteins; C103587:recombinant FVIIa; D015942:Factor VIIa