title: Splenectomy in two children with autoimmune lymphoproliferative syndrome and massive splenomegaly.
abstract: Treatment of patients with ALPS has varied but presently there is no consensus about the optimal therapy. Splenectomy is an option but data regarding the postsplenectomy outcome in pediatric ALPS patients remain very limited. We present two children who suffered from anemia and physical discomfort from the large spleen. Both patients underwent uneventful splenectomy and experienced significant improvement in cytopenia, daily activity and well-being. Furthermore the youngest patient showed a significant catch-up growth. We conclude that in selected patients with marked splenomegaly and ALPS, splenectomy may be considered a treatment option.
authors.affiliation: Department of Pediatrics, Aarhus University Hospital Skejby, Aarhus, Denmark.
authors.forename: Mia
authors.initials: M
authors.lastname: Glerup
authors.forename: Thomas
authors.initials: T
authors.lastname: Thelle
authors.forename: Kirsten
authors.initials: K
authors.lastname: Grønbaek
authors.forename: Henrik
authors.initials: H
authors.lastname: Hasle
mesh_terms: D000293:Adolescent; D000744:Anemia, Hemolytic, Autoimmune; D005260:Female; D006128:Growth; D006801:Humans; D007223:Infant; D008232:Lymphoproliferative Disorders / Q000150:complications* / Q000276:immunology; D008297:Male; D013156:Splenectomy; D013163:Splenomegaly / Q000601:surgery*; D016896:Treatment Outcome