Acute splenic sequestration crises in Saudi children with sickle cell disease

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Sickle cell disease (SCD) is a common disease in the Eastern Province of Saudi Arabia. Twenty per cent of the population have sickle cell trait and 1.75% have SCD. In the first 2 years of life, infection, hand-foot syndrome and acute splenic sequestration crisis (ASSC) are the most common complications of this disease. The classical characteristics of an ASSC, the subject of this paper, are sudden and rapid enlargement of the spleen, secondary to the massive pooling of red blood cells in the splenic sinusoids in a functioning spleen. Less common minor attacks of ASSC have been described recently. An ASSC is one of the most common causes of death in infants with SCD. The underlying cause and the precipitants of attacks of ASSC remain unknown. Seventeen children with ASSC were seen. Clinically, they had minor ASSC; three developed hypersplenism and four underwent splenectomy for recurrent attacks of ASSC. None of these 17 children had the classical ASSC described in black children where peripheral circulatory shock is encountered.