RT Book, Section A1 Bissonnette, Bruno A1 Luginbuehl, Igor A1 Engelhardt, Thomas SR Print(0) ID 1164072357 T1 Hemoglobin E Disease T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e YR 2019 FD 2019 PB McGraw-Hill Education PP New York, NY SN 9781259861789 LK accessanesthesiology.mhmedical.com/content.aspx?aid=1164072357 RD 2024/03/28 AB Hemoglobin (Hb) E is one of the world’s most common and important mutations. The clinical presentation of E β-thalassemia is associated with complications that may lead to serious morbidity and mortality risks. Patients are at high risk for thromboembolism secondary to a hypercoagulable state following resection of the spleen. Hemosiderosis is associated with cardiopulmonary disease such as cardiomyopathy, pulmonary hypertension, and right heart failure. Thromboembolism and hemolysis-induced nitric oxide deficiency result in pulmonary vascular injury and subsequent cardiac disease. Both transfused and nontransfused patients with Hb E β0-thalassemia are at risk for life-threatening complications and should be followed by a multidisciplinary team. Other organs that may be involved include endocrine, pulmonary, hepatobiliary, and the skeletal system. The use of hydroxyurea may have some benefit as it is shown to have improved 40% of patients.