La séquestration splénique aiguë : une complication rare et grave des syndromes drépanocytaires majeurs de l'adulte. À propos d'un cas

Abstract

Acute splenic sequestration, a well-recognized complication of sickle cell syndromes, is characterized by a sudden decrease in haemoglobin concentration and marked painless splenomegaly. We report a case illustrating the outcome and the treatment options of this complication. A 45-year old homozygous woman developed acute splenic sequestration with severe anemia. Red blood cells transfusion led to transient improvement but a relapse-required splenectomy. Long-term outcome was favorable. Acute splenic sequestration is a severe complication mainly observed in children. Despite the severity of this complication, prompt diagnosis and appropriate therapy, and particularly red blood cells transfusions, led to a complete recovery. Splenectomy is required in the more severe form of the disease.