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.
Grover
R
R
St. Luke's-Roosevelt Hospital Center, Comprehensive Sickle Cell Program, Columbia Presbyterian Medical Center, New York, New York.
Wethers
D L
DL
D000208:Acute Disease; D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D013158:Splenic Diseases / Q000209:etiology / Q000628:therapy*