Thalassemia in the emergency department: special considerations for a rare disease
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REVIEW ARTICLE
Thalassemia in the emergency department: special considerations for a rare disease Antoine N. Saliba 1 & Ali Atoui 2 & Mohamad Labban 2 & Hani Hamade 3 & Rayan Bou-Fakhredin 4 & Afif Mufarrij 3 & Ali T. Taher 4 Received: 4 June 2020 / Accepted: 29 June 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract 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. Keywords Thalassemia . Emergency department . Iron overload . Resuscitation
Introduction The thalassemias constitute a variable group of anemias characterized by a defect in the synthesis of one or more of the globin subunits of hemoglobin [1]. The hallmarks of this group of disorders include imbalance in the α/β-globin chain ratio, ineffective erythropoiesis, chronic hemolytic anemia, and iron overload [2]. Thalassemic disorders were historically clustered in the Mediterranean region and the Middle East, sub-Saharan Africa, and East and Southeast Asia [3]. Modern migration patterns have altered the epidemiology of thalassemia to include Northern and Western Europe, North America, and Australia [2–5].
* Ali T. Taher [email protected] 1
Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN, USA
2
Department of Internal Medicine, American University of Beirut Medical Center, Beirut, Lebanon
3
Department of Emergency Medicine, American University of Beirut Medical Center, Beirut, Lebanon
4
Division of Hematology and Oncology, Department of Internal Medicine, American University of Beirut Medical Center, Beirut, Lebanon
The thalassemia spectrum is clinically divided into two main categories based on patients’ needs for blood transfusion [2, 6]. Patients with transfusion-dependent thalassemia (TDT) commonly present in early childhood with severe anemia that requires lifelong regular transfusion therapy for survival [2]. Patients with non-transfusion-dependent thalassemia (NTDT) present either later in childhood or in adulthood with mild or moderate anemia that only requires occasional or intermittent transfusions in certain clinical settings [2, 6]. Advances in understanding the pathophysiology of thalassemic disorders have contributed to the optimization of transfusion programs and the timely implementation of iron chelation ther
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