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splenic sequestration
Etiology:
- occurs in people with sickle cell disease
- often associated with viral or bacterial infections
- up to 20% may be associated with acute chest syndrome [2]
- may occur in people with hemoglobin SC disease (ICD10)
Epidemiology:
- more common in infants & young children since autoplenectomy often occurs with sickle cell disease
- recurrence in 50% of survivors of 1st episode
Pathology:
- occurs when large numbers of sickle cells become trapped in the spleen, causing it to suddenly enlarge
Clinical manifestations:
- splenomegaly
- sudden weakness, pallor, tachycardia, tachypnea
- abdominal fullness
Laboratory:
- complete blood count
- peripheral smear for sickle cells
- reticulocyte count for aplastic crisis
Complications:
- sudden & severe anemia
- hypovolemic shock
- death, mortality up to 20%
General
splenic disease
complication
References
- WebMD: Splenic Sequestration and Sickle Cell Disease
http://www.webmd.com/a-to-z-guides/splenic-sequestration-and-sickle-cell-disease
- Wang WC
SPLENIC SEQUESTRATION AND TRANSIENT APLASTIC CRISES
http://sickle.bwh.harvard.edu/spleen.html