Glanzmann's thrombasthenia proposed optimal management during surgery and delivery

Abstract

Glanzmann's thrombasthenia (GT) is an autosomal recessive disorder of platelet function. Conventional management is by platelet transfusion, given before invasive interventions. Alloimmunization resulting in platelet refractoriness and an unpredictable response to platelet infusion have provided particular management difficulties in the past. More recently recombinant (r)VIIa (Novoseven®) has a valuable role in the treatment of platelet function disorders. Treatment of a patient with GT during two pregnancies and spinal surgery is reported. An algorithm is presented to provide a structured and consistent approach to treatment.