[en] 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.
Disciplines :
Hematology Surgery Anesthesia & intensive care
Author, co-author :
WERTZ, Damien ; Centre Hospitalier Universitaire de Liège - CHU > Anesthésie et réanimation
Boveroux, Pierre ; Centre Hospitalier Universitaire de Liège - CHU > Hématologie biologique et immuno hématologie
PETERS, Pierre ; Centre Hospitalier Universitaire de Liège - CHU > Neurochirurgie
LENELLE, Jacques ; Centre Hospitalier Universitaire de Liège - CHU > Anesthésie et réanimation
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