Perioperative management of a patient with Bernard–Soulier syndrome that has a refractoriness to platelet transfusion

Abstract

A patient with Bernard–Soulier syndrome (BSS), characterized by extremely large platelets, thrombocytopenia and prolonged bleeding time underwent general anesthesia for platelet adhesion aberration. This patient has developed a refractoriness to platelet transfusion due to a frequent transfusion of platelets; however the operation was completed without abnormal bleeding by transfusing HLA-matched platelets during the procedure. BSS is a very rare syndrome so there are no well-defined protocols for the management of perioperative bleeding associated with this syndrome. In this report, we present the detail of the perioperative management for these patients with BSS who has a refractoriness to platelet transfusion.