First case report of perioperative management in a novel entity of macrothrombocytopenia: the autosomal dominant macrothrombocytopenia with platelet dysfunction associated with reduction of surface αIIbβ3

Abstract

Autosomal dominant macrothrombocytopenia with platelet dysfunction associated with reduction of surface αIIbβ3 (ADM) is an autosomal dominant form of Glanzmann’s thrombasthenia reported in few families in the literature. This case presents the first report of perioperative approach of a patient with ADM successfully submitted to a surgical procedure. The multidisciplinary approach including immunohaemotherapy consultation was considered the best clinical approach to avoid bleeding. The use of desmopressin and tranexamic acid in the preoperative period followed by desmopressin plus aminocaproic acid in the postoperative period showed to be safe and effective in the perioperative approach of the patient with ADM.