title: Retrospective evaluation of long-term efficacy and safety of splenectomy in chronic idiopathic thrombocytopenic purpura in children.
abstract: OBJECTIVE
  To review the long-term efficacy and safety of splenectomy in children with chronic idiopathic thrombocytopenic purpura (cITP).
  
  
  METHODS
  Data from 33 splenectomized children were retrospectively analysed (median follow up period: 18.8 y from the removal of the spleen). The median age of children at splenectomy was 12 y and the median ITP duration 3.3 y. Indications for splenectomy were: persistent severe thrombocytopenia with extensive purpura, epistaxis and/or gum bleeds, menorrhagia (n = 5) and severe or recurrent haemorrhage from various sites (n = 11).
  
  
  RESULTS
  Eighty-five per cent of the patients showed an excellent (n = 26) or partial response to splenectomy. Five children (15%), all females, failed to respond. Of the responders, 25% experienced a transient recurrence of thrombocytopenia within 6 mo to 4 y from splenectomy. The mortality rate due to severe sepsis was 3%. However, the majority of the splenectomized patients have not so far suffered any severe or mild bacterial infection, despite incomplete vaccination and/or antibiotic prophylaxis.
  
  
  CONCLUSIONS
  Splenectomy remains the only effective therapeutic modality for children with cITP, although it is associated with transient recurrence and rarely with post-splenectomy sepsis, which could be fatal. Nonetheless, splenectomy should be the last treatment option for the cITP patient, after all available therapeutic modalities have been exhausted and the child still remains profoundly thrombocytopenic and symptomatic.
authors.affiliation: Haemophilia and Haemostasis Unit, Aghia Sophia Children's Hospital, Athens, Greece. sofaronis@in.gr
authors.forename: S
authors.initials: S
authors.lastname: Aronis
authors.forename: H
authors.initials: H
authors.lastname: Platokouki
authors.forename: M
authors.initials: M
authors.lastname: Avgeri
authors.forename: H
authors.initials: H
authors.lastname: Pergantou
authors.forename: D
authors.initials: D
authors.lastname: Keramidas
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000601:surgery*; D012189:Retrospective Studies; D013154:Spleen / Q000601:surgery; D013156:Splenectomy; D013997:Time Factors; D016896:Treatment Outcome