Sickle Cell Disease

Annals in the Clinic

Sickle cell disease is an umbrella term for a group of hemoglobinopathies characterized by the presence of 2 β-globin gene mutations or deletions, at least 1 of which is the point mutation that leads to the production of hemoglobin S. Sickle cell disease is associated with hemolytic anemia, significant chronic end-organ damage, and early death. In high-income countries, at least 95% of children with the disease survive into adulthood, resulting in a growing population of affected adults with significant comorbidities, complex medical issues, and not enough specialists to provide care. Hydroxyurea is the primary therapy, and recent advances in understanding disease pathophysiology have led to new therapies; progress in bone marrow transplant and gene editing has resulted in cure in some patients. The purpose of this review is to provide an overview of the diagnosis, common acute and chronic clinical manifestations, and best practices for management of sickle cell disease.


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Annals in the Clinic

Annals In the Clinic is a monthly feature in Annals of Internal Medicine introduced in January 2007 that focuses on practical management of patients with common clinical conditions. It offers evidence-based answers to frequently asked questions about screening, prevention, diagnosis, therapy, and patient education and provides physicians with tools to improve the quality of care.