Splenectomy is successful in ∼70–80% of pediatric chronic immune thrombocytopenia (cITP) patients, although there was no data on its effectiveness in autoimmune hemolytic anemia (AIHA) and Evans syndrome (ES). The decision to undergo splenectomy in children is quite challenging due to the procedure’s irreversibility and the lack of predictability regarding the long-term effects.
For a study, researchers presented findings from the OBS’CEREVANCE cohort, which included French children suffering from autoimmune cytopenia (AIC) since 2004. The main endpoint was failure-free survival (FFS), which is commonly called the period between splenectomy and the start of second-line therapy (other than steroids and intravenous immunoglobulins) or death. They included 161 patients with a median (minimum-maximum) follow-up of 6.8 years (1.0-33.3) following splenectomy (cITP, n=120; AIHA, n=19; ES, n=22). The AIC subtype was not linked to the FFS.
Immunopathological manifestations (IMs) were found to be closely related to adverse results. Prior to splenectomy, an IM diagnosis was linked to a reduced FFS (adjusted by AIC subtype, hazard ratio [HR], 0.39; 95% CI, 0.21-0.72, P=.003). In addition to a reduced FFS (HR, 0.22; 95% CI, 0.12-0.39; P=2.8×10-7, adjusted for AIC subtype), an IM diagnosis was linked to a much higher risk of thrombosis and recurring or severe bacterial infections at any moment during the follow-up.
Finally, the findings suggested the search for related IMs when planning a splenectomy in order to refine the risk-benefit ratio. Following the surgery, monitoring IMs aids in identifying patients who are at a higher risk of adverse outcomes.
