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Overview of the clinical manifestations of sickle cell disease

Elliott P Vichinsky, MD
Section Editor
Stanley L Schrier, MD
Deputy Editor
Jennifer S Tirnauer, MD


The sickle point mutation in the beta globin gene results in the production of sickle hemoglobin, which is less soluble than normal fetal or adult hemoglobin. Sickle cell disease (SCD) refers to any one of the syndromes in which the sickle mutation is co-inherited with a mutation at the other beta globin allele that reduces or abolishes normal beta globin production. These include sickle cell anemia (homozygous sickle mutation), sickle beta thalassemia, hemoglobin SC disease, and others.

The clinical manifestations of SCD are protean. The major features are related to hemolytic anemia and vasoocclusion, which can lead to acute and chronic pain and tissue ischemia or infarction. Splenic infarction leads to functional hyposplenism early in life, which in turn increases the risk of infection. These complications have a major impact on morbidity and mortality.

This topic review presents an overview of the major clinical manifestations of SCD. Each of these is discussed in more detail in separate topic reviews mentioned herein.

General overviews of the pathophysiology, diagnosis, and management of SCD are also presented separately.

Pathophysiology – (See "Mechanisms of vasoocclusion in sickle cell disease" and "Clinical variability in sickle cell anemia" and "Sickle hemoglobin polymer: Structure and functional properties".)


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