[Role of splenectomy in XXXX in its accelerated phase].

@entity1 @entity3329 @entity3917 @entity706 @entity55 @entity828 @entity465 @entity4068 @entity6118 @entity13277 @entity346 @entity3043
@entity13277 is a rare @entity465 , due to defective granulocyte activity. The syndrome is characterized by large inclusion bodies in the leukocytes, @entity4068 , @entity3917 , @entity346 , and recurrent infections. Some @entity1 develop @entity828 , @entity3329 , @entity3043 and widespread organ infiltrates with mononucleated cells. This phase is called "accelerated (or @entity706 ) phase". A 5 years old @entity1 with C.H.S. in accelerated phase received initially medical treatment without improvement. A splenectomy was performed to remove the @entity6118 and the mechanical compression of the spleen on the gut. Few days after the splenectomy the fever and the @entity3043 disappeared. The pathological examination of the spleen showed multiple intraparenchymal abscesses. Unfortunately, six months after the operation, she died after an acute episode of @entity55 , with normal hematological pattern. The splenectomy may play a role in the "accelerated phase" of C.H.S., but new treatments (bone marrow transplantation) are necessary to remove the basic disease.
