The cause of this syndrome is unclear; it may be seen in the setting of concurrent bacterial or viral infection.
Diagnostic criteria
The diagnostic criteria for acute splenic sequestration include an acutely enlarging spleen, a decrease in hemoglobin level of at least 2 g per dL below baseline, thrombocytopenia often with a platelet count <100,000 per mm,3 leukopenia, and evidence of bone marrow compensation with reticulocytosis.
In its most severe form, acute splenic sequestration results in life-threatening anemia, hypovolemia, and shock. Decreases in the levels of hemoglobin <4 g per dL are associated with 35% mortality rates. It is estimated that as much as 50% of the patient's red cells can be sequestered in the spleen. Approximately 50% of patients who survive an episode of acute splenic sequestration will experience a recurrence.
Mangement
Emergency management is aimed at restoring circulatory blood volume and hemodynamic stability. Although
therapy may begin with crystalloid resuscitation, red blood cell transfusions must be given promptly.
Long-term management is complicated because there is a high rate of recurrence of either acute or subacute sequestration. Splenectomy has been advocated when recurrent episodes occur.
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