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
• 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%

More general terms

• splenic disease
• complication