title: Splenectomy in children with idiopathic thrombocytopenic purpura: A prospective study of 134 children from the Intercontinental Childhood ITP Study Group.
abstract: BACKGROUND
  Splenectomy is an effective procedure for children and adults with severe or refractory idiopathic thrombocytopenic purpura (ITP). Data regarding pediatric patients are limited.
  
  
  METHODS
  Sixty-eight Intercontinental Childhood ITP Study Group (ICIS) investigators from 57 institutions in 25 countries participated in a splenectomy registry. Data from 153 patients were submitted, of whom 134 had a splenectomy and were analyzed.
  
  
  RESULTS
  The median age at splenectomy was 11.8 (2.7-20.7) years. The median postsplenectomy follow-up was 2.0 (0.1-4.5) years. Pre-splenectomy vaccination was not administered in 21 children (15.7%). Open and laparoscopic splenectomy procedures were performed in 67 and 65 evaluable children, respectively. Surgical technique was not reported in two children. Overall immediate platelet response to splenectomy was achieved in 113 patients (86.3%). Eighty percent of responders maintained their status of response during the following 4 years. Older age, longer duration of ITP, and male gender correlated with a complete response. Post-splenectomy sepsis was reported in seven patients without lethal outcome, although sepsis might be differently defined at participating institutions.
  
  
  CONCLUSIONS
  Splenectomy is effective in children with ITP. Management varies greatly in different institutions. These Registry data may serve as a basis for future clinical trials to assess the indication and timing of splenectomy.
authors.affiliation: University Children's Hospital, Basel, Switzerland. Thomas.Kuehne@ukbb.ch
authors.forename: Thomas
authors.initials: T
authors.lastname: Kühne
authors.forename: Victor
authors.initials: V
authors.lastname: Blanchette
authors.forename: George R
authors.initials: GR
authors.lastname: Buchanan
authors.forename: Ugo
authors.initials: U
authors.lastname: Ramenghi
authors.forename: Hugo
authors.initials: H
authors.lastname: Donato
authors.forename: Rienk Y J
authors.initials: RY
authors.lastname: Tamminga
authors.forename: Johannes
authors.initials: J
authors.lastname: Rischewski
authors.forename: Willi
authors.initials: W
authors.lastname: Berchtold
authors.forename: Paul
authors.initials: P
authors.lastname: Imbach
mesh_terms: D000293:Adolescent; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D010976:Platelet Count; D011446:Prospective Studies; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000097:blood* / Q000150:complications / Q000601:surgery*; D020127:Recovery of Function; D012042:Registries; D012074:Remission Induction; D018805:Sepsis / Q000097:blood / Q000209:etiology; D012737:Sex Factors; D013156:Splenectomy