Management of acute splenic sequestration crisis in sickle cell disease

Acute splenic sequestration crisis (ASSC) is a significant cause of early morbidity in children with sickle cell disease. With timely diagnosis and prompt transfusion, the outcome is good. However, recurrences are frequent, and both splenectomy and long-term transfusion therapy have been advanced as appropriate preventive approaches. We describe the diagnosis, management, and course of 15 patients with ASSC followed at the St. Luke's-Roosevelt Hospital Center Comprehensive Sickle Cell Program. Based on our experience, we recommend surgery after the first episode of ASSC in the child 5 years of age and older and the choice of a year or more of long-term transfusion therapy for the child under 3. With intensive education of the parents, and with close observation and individualized management of the patient, the overall prognosis may be improved.