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Overview of variant sickle cell syndromes

Elliott P Vichinsky, MD
Section Editor
Donald H Mahoney, Jr, MD
Deputy Editor
Jennifer S Tirnauer, MD


Variant sickle cell syndromes include any hemoglobinopathy in which the sickle mutation is inherited in combination with another globin gene mutation (eg, alpha globin, beta globin, gamma globin). These syndromes may have different clinical severity compared with homozygous sickle mutation (HbSS).

This topic presents an overview of the variant sickle cell syndromes and their clinical features.

Related subjects including the diagnosis of sickle syndromes, clinical manifestations, and management, as well as sickle cell trait (generally a benign carrier state) are discussed separately.

Prenatal testing – (See "Prenatal screening and testing for hemoglobinopathy".)

Diagnosis – (See "Diagnosis of sickle cell disorders".)


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Literature review current through: Sep 2016. | This topic last updated: Mar 18, 2016.
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