Emergencies in Patients With Inherited Hemoglobin Disorders—An Emergency Department Perspective

Hemoglobin (Hb) disorders presenting to the emergency department (ED) can be a diagnostic and management problem if not properly recognized. It is important to understand the nomenclature and pathophysiology of these entities. Common Hb disorders presenting to the ED include sickle cell disease, thalassemias, and methemoglobinemia. Complications of sickle cell disease comprise the majority of illness from Hb disorders seen in the ED or outpatient setting. These complications include pain crisis, sepsis, acute chest syndrome, and cerebrovascular accidents. Rapid diagnosis and treatment are necessary to minimize morbidity and mortality. In a case-based format, the clinical presentation, assessment, and management of the above will be discussed to help clinicians practice in an evidence-based fashion.

Keywords: hemoglobin disorders, sickle cell disease, thalassemias, methemoglobinemia