Acute Splenic Sequestration Crises (ASSC) in Young Children with Sickle Cell Anemia

Abstract

Acute splenic sequestration crises (ASSC) is a potentially life-threatening com plication in young children with sickle cell anemia. The clinical picture includes huge splenomegaly accompanied by signs and symptoms of acute circulatory insufficiency caused by profound anemia. The authors describe 20 such episodes in 14 children aged six to 55 months. Rapid transfusion of packed erythrocytes produced rapid and dramatic improvement. The spleens became nonpalpable in seven to ten days following transfusion. Four children died, four had one or more recurrences, four have splenic involution, and two were splenectomized. The recurrences were within four months of the previous ASSC. "Our current recommendation is to consider splenectomy only after at least two ASSCs."