Splenic sequestration and sickle cell disease

Splenic sequestration occurs in people with sickle cell disease when large numbers of sickled red blood cells become trapped in the spleen, causing it to suddenly enlarge. This condition most commonly occurs in children age 6 months to 2 years and often follows or accompanies a simple respiratory infection.¹ Without emergency medical care, splenic sequestration can cause death in a matter of hours.

Splenic sequestration causes sudden and severe anemia, with symptoms of sudden weakness, pale lips, rapid breathing, excessive thirst, abdominal pain, and rapid heartbeat.

Parents of babies and young children with sickle cell disease are advised to monitor their children for spleen enlargement, as demonstrated by a health professional. A suddenly enlarged spleen requires emergency medical care.

Citations

1. Greer JP, Lukens JN (2004). Sickle cell anemia and other sickling syndromes. In JP Greer et al., eds., Wintrobe's Clinical Hematology, 11th ed., vol. 1, pp. 1263–1311. Baltimore: Williams and Wilkins.