Treatment of bleeding in an individual with Glanzmann thrombasthenia (GT)

Patients undergoing surgery (emergency or elective) may use a similar approach. Refractoriness to platelet transfusion can develop in an individual with GT if they are exposed to platelets and develop antibodies to platelet GPIIb/IIIa and/or to platelet HLA antigens. In such cases, platelet transfusions may be ineffective for treating severe bleeding or major surgery. The benefit of rFVIIa must be balanced against the possible risks of VTE and arterial thrombosis, which are thought to be very low in GT but may occur, especially in individuals with underlying risk factors for venous or arterial thromboembolic disease (eg, vascular disorders, prior VTE). Refer to UpToDate for details.

GT: Glanzmann thrombasthenia (hereditary deficiency of the platelet glycoprotein alpha_IIbbeta₃ integrin, previously called the glycoprotein [GP]IIb/IIIa receptor); HLA: human leukocyte antigen; RBCs: red blood cells; rFVIIa: recombinant activated factor VII; VTE: venous thromboembolism.

* Platelet transfusion and rFVIIa are often given together despite lack of high quality evidence that the combination is more effective than either given alone. Some individuals may use rFVIIa to avoid platelet transfusions, which carry a risk of developing antibodies to platelet GPIIb/IIIa and/or HLA or to treat bleeding if these antibodies have already developed. NovoSeven is approved for patients with GT at a dose of 90 mcg/kg. The dose is repeated approximately every 90 to 120 minutes until bleeding has stopped. Following surgery, dosing every 2 to 6 hours is reasonable until the concerns about hemostasis have resolved. Refer to product information for additional details.

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Treatment of bleeding in an individual with Glanzmann thrombasthenia (GT)