A 47-year-old male with a history of warm antibody autoimmune hemolytic anemia presented with recurrent hemolysis 2 months after laparoscopic splenectomy. On presentation, his hemoglobin was 6.9 g/dL with normal leukocyte and platelet counts. The blood smear revealed 1+ spherocytes without microangiopathy. The patient stated that he had dark brown urine on the day before presentation and a chemistry panel showed unconjugated hyperbilirubinemia, elevated lactate dehydrogenase, and undetectable haptoglobin. A direct antiglobulin test was positive for IgG. His recurrent hemolytic anemia was refractory to treatment with corticosteroids and intravenous immune globulin.
A 99mtechnetium-labelled heat-damaged erythrocyte scan suggested presence of several foci of accessory splenic tissue in the left upper quadrant (top image). A subsequent contrast-enhanced computed tomography confirmed multiple distinct accessory splenules (arrows) in the left retrocolic gutter (lower image series). The patient underwent laparoscopic removal of the splenules and experienced improvement in his hemoglobin level and transfusion independence but continued to show biochemical evidence of hemolysis. After initiating treatment with rituximab, his hemolysis resolved by all measures.1
Recurrent immune-mediated hemolysis after splenectomy may indicate compensatory hypertrophy of accessory splenic tissue. Heat-damaged erythrocytes become entrapped in reticuloendothelial tissue, thus providing a useful clinical test for identifying accessory splenules.