A Patient with Glanzmann’s Thrombasthenia for Emergent Abdominal Surgery

Abstract

In Brief Glanzmann’s thrombasthenia is a rare autosomal recessive disease characterized by potentially major mucocutaneous complications and nose bleeds. It is considered hazardous for these surgical patients to conceive, with a high risk of urgent surgery. The treatment of bleeding or prevention of hemorrhage for surgery or invasive procedures is based on platelet transfusion. However, platelet transfusions may be responsible for the development of alloimmunization, with a high risk of future platelet refractoriness. We report a surgical case of Glanzmann’s thrombasthenia complicated by nasopharyngeal bleeding and managed with platelet transfusions, recombinant activated factor VII, and postoperative airway management in the intensive care unit. IMPLICATIONS: We describe the management of an emergent surgical patient with Glanzmann’s thrombasthenia and nasopharyngeal bleeding requiring platelets and recombinant activated factor VII infusions. These patients may need postoperative intensive care.