Clinical Scenario

Glanzmann's thrombasthenia is a rare inherited platelet function disorder characterised by impaired platelet aggregation. This protocol addresses the management of moderate to severe bleeding episodes, as well as the perioperative setting in patients undergoing minor or major surgical procedures.

Treatment Approach

For moderate to severe bleeding and surgical procedures, the approach involves a recombinant bypassing agent. Depending on the clinical context, this agent may be used alone or in combination with adjunct hemostatic therapy.

The complete regimen — including sequencing, perioperative protocol, and combination strategies — is available in the full structured protocol below →

References

rFVIIa Moderate to severe bleeding
Alone for minor surgical procedures or in combination with antifibrinolytics and/or platelet transfusions for major procedures and severe or life-threatening bleeding
Typical doses for acute bleeding are 90 mcg/kg intravenous (IV) every 2-6 hours until hemostasis is achieved (at least 3 doses).
Perioperative dosing is 90 mcg/kg immediately before surgery and every two hours during the procedure, with doses every 2-6 hours postoperatively to prevent post-surgical bleeding.

DOI: 10.3324/haematol.2018.214239

View source ↗

Treatment of Glanzmann's Thrombasthenia in Moderate to Severe Bleeding and Surgical Settings

Clinical Scenario

Treatment Approach

Treatment Goal

References