PB1396 - Successful hip replacements in a Glanzmann thrombasthenia patient with anti-platelet antibodies and platelet- refractory disease using rFVIIa: a case report

Background: Glanzmann thrombasthenia (GT) is rare congenital disorder characterized by platelet disfunction due to deficit of αIIbβ3 integrin on platelet membrane and various bleeding manifestations. Antifibrinolytic therapy, recombinant activated factor VII(rFVIIa) and platelet transfusions are mainstay of treatment, although the latter might trigger allo anti-platelet antibodies formation resulting in platelet-refractory disease. Management of GT patients remains complex, particularly in the context of trauma, child delivery, and perioperative care. Recombinant FVIIa is indicated for use in patients with anti-platelet antibodies and platelet-refractory disease either to prevent or to treat bleeding. Regimens for surgical prophylaxis, including dosage, dose intervals and duration of treatment, remain undefined.

Aims: We report a case of successful use of rFVIIa for two major surgical procedures in one patient with no adverse events.

Methods: Case report

Results: A 48-year-old female, refractory to platelet transfusions, required both hip replacements (left hip in March 2019, right hip in April 2021) due to degenerative changes. Decision to use bolus injections of rFVIIa and tranexamic acid for surgery was made.
On both occasions she received initial bolus dose of rFVIIa 90 mcg/kg on the day of surgery preoperatively and then every 2 hours in first 24 hours. The dosage of rFVIIa remained unchanged throughout treatment. Daily usage of rFVIIa and dose intervals are depicted in Table 1.
Because of wound oozing, patient required more doses of rVIIa for right hip replacement (7.43 mg/kg vs. 5.16 mg/kg, respectively).
There were no bleeding complications and no thrombotic events. Patient received no anticoagulant prophylaxis.

Conclusion(s): Our case demonstrates safe use of recombinant FVIIa for major surgical procedures in a GT patient refractory to platelet transfusions. The variations in cumulative dose used for the same type of surgery shows variability of clinical course in same patient. More data is needed to provide general recommendations in these patients.