Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN, USA.
Department of Internal Medicine, American University of Beirut Medical Center, Beirut, Lebanon.
Ann Hematol. 2020 Sep;99(9):1967-1977. doi: 10.1007/s00277-020-04164-6. Epub 2020 Jul 3.
Thalassemia is characterized by a defect in the synthesis of one or more of the globin subunits of hemoglobin. This defect results in imbalance in the α/β-globin chain ratio, ineffective erythropoiesis, chronic hemolytic anemia, and iron overload. With advances in diagnosis, treatment, and transfusion support, the prognosis of patients with thalassemia has improved over the past few decades. An increasing number of patients with thalassemia is living with long-term complications, including cardiomyopathy, chronic liver disease, endocrinopathy, and infections. In this paper, we review common complications that bring the patient with thalassemia to urgent or emergent medical attention. We also discuss the aspects of emergency care that are most relevant while caring for the patient with thalassemia in the emergency department.
地中海贫血的特征是血红蛋白的一个或多个球蛋白亚基的合成缺陷。这种缺陷导致α/β-球蛋白链比例失衡、无效的红细胞生成、慢性溶血性贫血和铁过载。随着诊断、治疗和输血支持的进步，过去几十年来地中海贫血患者的预后得到了改善。越来越多的地中海贫血患者患有长期并发症，包括心肌病、慢性肝病、内分泌疾病和感染。本文回顾了常见的并发症，这些并发症使地中海贫血患者需要紧急或紧急医疗关注。我们还讨论了在急诊科照顾地中海贫血患者时最相关的紧急护理方面。
