Revisiting Splenectomy in Childhood Immune Thrombocytopenic Purpura in the Era of New Therapies: The French Experience
Nathalie Aladjidi1,2*, Raoul Santiago1,2, Corinne Pondarré3, Anne Lambilliotte4, Guy Leverger5, Claire Godard Sebillotte6,
Vincent Barlogis7, Pierre Rohrlich8, Marlène Pasquet9, Sophie Bayart10, Dominique Plantaz11, Patrick Lutz12, Karine de Bosredon13, Aude Marie-Cardine14, Corinne Guitton15, Patrick Boutard16, Martine Munzer17, Jean-Louis Stephan18, Thierry Leblanc19 and Y. Perel1,2
- Corresponding Author:
- Nathalie Aladjidi
Pediatric Hematology Unit
Centre de Référence National des Cytopénies Auto-immunes de l’Enfant(CEREVANCE)
Hôpital des Enfants, Hôpital Pellegrin, CHU Bordeaux
Place Amélie Raba Léon, 33 000 Bordeaux, France
Tel: 00 33 5 57 82 02 61
Fax: 00 33 5 57 82 02 79
E-mail: [email protected]
Received Date: April 20, 2012; Accepted Date: May 16, 2012; Published Date: May 22, 2012
Citation: Aladjidi N, Santiago R, Pondarré C, Lambilliotte A, Leverger G, et al. (2012) Revisiting Splenectomy in Childhood Immune Thrombocytopenic Purpura in the Era of New Therapies: The French Experience. J Blood Disord Transfus S3:003. doi: 10.4172/2155-9864.S3-003
Copyright: © 2012 Aladjidi N, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Objective: While splenectomy is the gold standard treatment for refractory primary immune thrombocytopenia (ITP) in adult, its place remains debated in children. The French Rare Disease Plan provided us the opportunity to conduct a collaborative study of the efficiency and tolerance of this procedure in childhood ITP.
Patients and methods: A retrospective study was conducted in France in order to identify children with ITP treated with splenectomy during a 9-year period. A total of 78 children were included. Data from the ongoing CEREVANCE national cohort of childhood auto-immune cytopenia in 30 units were reviewed and completed by a direct contact with the referent physicians. International terminology for response definition was used. Relapsefree survival was assessed by the Kaplan-Meier method.
Results: The median ages at ITP diagnosis and splenectomy were 9.6 and 12.4 years respectively. The median duration of ITP before splenectomy was 24 months (1-162); 62 children had chronic ITP. The median number of treatment lines before splenectomy was 2 (1-7). Laparoscopy was used in 81% of cases. Four children underwent immediate surgical complications. With a median follow-up of 41 months, complete remission (CR) was maintained at the latest news in 84% of children. CR was obtained in 77% of cases with intra-splenic platelets destruction, and in no case with non-splenic destruction (p=0.11). Using a very strict definition for relapse, the 5-year relapse-free survival was 51% [IC95% 37-64]. No death or overwhelming sepsis was reported.
Conclusions: In this national study with a long term follow up, the excellent benefit/risk ratio of splenectomy for refractory ITP confirms that in skilled and concerted teams, the procedure is still at the forefront of curative treatments. Isotopic evaluation is of value but other prognostic factors for CR are to be determined. Lifelong survey of potential infectious and thrombotic risk at adult age has to be coordinated by the referring physician. The place for other therapeutic options, in order to postpone as late as possible the splenectomy in childhood ITP is now to be determined.