Thalassemia in the emergency department: special considerations for a rare disease
- 3 July 2020
- journal article
- review article
- Published by Springer Science and Business Media LLC in Annals of Hematology
- Vol. 99 (9), 1967-1977
- https://doi.org/10.1007/s00277-020-04164-6
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
This publication has 80 references indexed in Scilit:
- Initial assessment and treatment with the Airway, Breathing, Circulation, Disability, Exposure (ABCDE) approachInternational Journal of General Medicine, 2012
- Part 8: Adult Advanced Cardiovascular Life SupportCirculation, 2010
- Redefining thalassemia as a hypercoagulable stateAnnals of the New York Academy of Sciences, 2010
- The inherited diseases of hemoglobin are an emerging global health burdenBlood, 2010
- Magnetic resonance evaluation of hepatic and myocardial iron deposition in transfusion‐independent thalassemia intermedia compared to regularly transfused thalassemia major patientsAmerican Journal of Hematology, 2009
- Cardiac iron across different transfusion-dependent diseasesBlood Reviews, 2008
- Fracture prevalence and relationship to endocrinopathy in iron overloaded patients with sickle cell disease and thalassemiaBone, 2008
- Duration of hypotension before initiation of effective antimicrobial therapy is the critical determinant of survival in human septic shock*Critical Care Medicine, 2006
- A prospective study to determine the frequency and clinical significance of alloimmunization post‐transfusionBritish Journal of Haematology, 1995
- Late Cardiac Complications of Chronic, Severe, Refractory Anemia with HemochromatosisCirculation, 1964