Revisiting Splenectomy in Childhood Chronic Immune Thrombocytopenia At the Era of New Therapies: The French Experience

While splenectomy is the gold standard treatment for adult's refractory primary immune thrombocytopenia (ITP), its place remains debated in children. The Rare Disease Plan gave us the opportunity to conduct a French collaborative work aiming to study efficiency, tolerance and prognostic factors associated with success of this procedure in childhood ITP.

A retrospective study was conducted in France, of less than 18 years old children and adolescents who underwent a splenectomy for ITP during a 9-year period (2000–2009). Data from both the CEREVANCE national cohort and systematic requests to 30 paediatric units were reviewed. Medical data and procedures were checked from patients' medical records. Complete remission (CR) was defined by platelet count of more than 100 G/L, continuous complete remission (CCR) was defined as CR with no relapse or treatment for at least one year (Aladjidi et al, Haematologica 2011). Relapse-free survival was assessed by Kaplan Meier method.

For 78 children in 16 units, the median ages at diagnosis of ITP and at splenectomy were respectively 9.6 (0.8–16.5) and 12.4 (3.5–17.4) years. The median duration of ITP before splenectomy was 24 months (1–162), 62 children had chronic ITP of more than 12 months duration. The surgical procedure was laparoscopy in 81% of children. Four patients experienced post-operative complications: 1 severe intra-abdominal hemorrhage requiring open surgery twelve hours after laparoscopy, 1 pleural and peritoneal hemorrhage after laparotomy, 1 mesenteric and splenic thrombosis and 1 pulmonary atelectasia. With a median follow up of 41 months (1–109), CR and CCR were achieved respectively for 83% and 75% of 62 children with chronic ITP. No overwhelming sepsis was reported during the follow-up. Isotopic platelet studies were available in 10 of the 16 units and assessed in 30 of the 44 concerned children. CR was obtained in 77% of 26 cases with predominant splenic destruction, and in 0% of 2 cases with non splenic destruction. Five-year relapse-free survival was 35% in children of less than 10 years old and 81% in children of more than 10 years old at initial diagnosis of ITP (p=0.0002).

This collaborative national study confirms the excellent benefit-risk ratio of splenectomy for refractory chronic ITP, especially in older children. Even if our own results are limited by the small number of patients, prior isotopic platelet studies is useful when locally available. Long term respect of anti-infectious measures is recommended.

Acknoledgements to the Association Française pour le Syndrome d'Evans (AFSE), the GIS - Institut des Maladies Rares - INSERM, and the French Health Ministry (Programme Hospitalier de Recherche Clinique 2005, Rare Diseases Plan 2007).

No relevant conflicts of interest to declare.