The pathophysiology of the sickle hemoglobinopathies and implications for perioperative management

Patients with sickle hemoglobinopathies are at risk for unique postoperative complications and increased mortality. Understanding the pathophysiology of these disorders is important for safe perioperative management. Because there is no animal model for sickle cell disease, understanding has progressed through in vitro studies and clinical observations. This review describes the clinical manifestations of the sickle hemoglobinopathies with special emphasis on current knowledge of pathophysiology. It also discusses issues of preoperative screening, transfusion, and postoperative care.