Acute splenic sequestration in children with sickle cell disease - an overview Acute splenic sequestration (ASS) is a life-threatening complication of sickle cell disease (SCD). The condition is important to recognize due to the fact that it can occur with previously unknown disease. ASS is one of the most common causes of death in children with SCD and is the result of blood suddenly getting congested in the spleen, resulting in splenomegaly, acute anemia, and hypovolemic shock. Timely and appropriate treatment is essential in preventing death. Episodes of ASS before one year of age are associated with a higher risk of recurrence. There is no established effective treatment for recurrent ASS; however, there is evidence that all children with SCD should be treated with hydroxyurea. In Sweden, our recommendation is to evaluate the indications for splenectomy after the first episode of ASS. Allogeneic hematopoietic stem cell transplantation (HSCT) is the only curative treatment, and all children with SCD should be evaluated with regard to the potential success of HSCT. This article presents an overview of the condition with Swedish recommendations.