Thalassemia in the emergency department: special considerations for a rare disease

Ann Hematol. 2020 Sep;99(9):1967-1977. doi: 10.1007/s00277-020-04164-6. Epub 2020 Jul 3.

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: Emergency department; Iron overload; Resuscitation; Thalassemia.

Publication types

  • Review

MeSH terms

  • Betacoronavirus
  • Blood Transfusion / methods
  • Blood Transfusion / trends
  • COVID-19
  • Cardiomyopathies / diagnostic imaging
  • Cardiomyopathies / epidemiology
  • Cardiomyopathies / therapy
  • Coronavirus Infections / diagnostic imaging
  • Coronavirus Infections / epidemiology
  • Coronavirus Infections / therapy
  • Diagnosis, Differential
  • Emergency Medical Services / methods
  • Emergency Medical Services / trends*
  • Emergency Service, Hospital / trends*
  • Humans
  • Liver Diseases / diagnostic imaging
  • Liver Diseases / epidemiology
  • Liver Diseases / therapy
  • Pandemics
  • Pneumonia, Viral / diagnostic imaging
  • Pneumonia, Viral / epidemiology
  • Pneumonia, Viral / therapy
  • Rare Diseases / diagnostic imaging*
  • Rare Diseases / epidemiology
  • Rare Diseases / therapy*
  • SARS-CoV-2
  • Thalassemia / diagnostic imaging*
  • Thalassemia / epidemiology
  • Thalassemia / therapy*