Recurrent splenic sequestration in a Kuwaiti boy with sickle cell disease

ABSTRACT

Children suffering from sickle cell disease are susceptible to different types of crises, such as acute vaso-occlusive, haemolytic, aplastic and sequestration crises. The classic acute sequestration crisis is a serious condition usually affecting black children, in which large amounts of blood are suddenly pooled into the spleen. The spleen suddenly enlarges markedly and signs of circulatory collapse rapidly develop, leading to death unless an emergency splenectomy is performed. Minor recurrent sequestration episodes have been recently reported in patients with sickle cell disease and a high fetal haemoglobin concentration. These episodes are of a milder presentation and outcome can be managed with repeated blood transfusions and later, elective splenectomy In this case report, we describe an Arab Kuwaiti boy with recurrent splenic sequestration who was treated with elective splenectomy, and propose a policy for the management of such patients