In infants and children with sickle cell or other unstable hemoglobinopathy, the spleen may suddenly enlarge due to sequestration, with accompanying anemia and thrombocytopenia. This may be sufficient to cause hypotension and even death.
Features of acute splenic sequestration in patients with sickle cell disease (after Rosse et al):
(1) sudden massive splenic enlargement: The spleen is easily palpable and tender, extending well below the left costal margin.
(2) decrease in hemoglobin at least 2 g/dL below baseline values
(3) thrombocytopenia, usually with values to 100,000 per µL
(4) reticulocytosis
(5) "overshoot" in hemoglobin and hematocrit after red blood cell transfusion, with higher values than expected from the amount of blood infused, due to release of blood from the spleen
In the severely affected child with hypotension and shock, emergency red blood cell transfusion can be lifesaving. Care must be taken not to over-transfuse, since the release of red blood cells from the spleen following transfusion must be considered.
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