Transfusion-Reversible Functional Asplenia in Young Children with Sickle-Cell Anemia

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Abstract
We recently defined functional asplenia in children with sickle-cell anemia as impaired splenic reticuloendothelial function despite clinical enlargement of the organ. In three children infusions of fresh plasma did not restore function, indicating that functional asplenia probably is not a consequence of a contracted plasma volume or deficient opsonins or other humoral factors. Transfusions of normal red cells were given, and in five young children, splenic function was restored. Seri al studies suggested that a level of normal red cells of about 50 per cent was necessary for function. The best explanation for functional asplenia is that the high viscosity of sickle-cell blood causes a diversion of splenic blood flow through intrasplenic shunts, thus bypassing the phagocytic reticuloendothelial elements of the organ. When sickled cells are replaced by normal red cells through transfusion, splenic circulation and function are temporarily restored.