Early splenectomy in a large cohort of children with sickle cell anemia: risks and consequences

Abstract

In children with sickle cell anemia (SCA), early splenic complications can mandate splenectomy but the benefit-to-risk ratio and the age at which splenectomy may be safely performed remain unclear. To address this question, we analyzed the rate of post-splenectomy events in children with SCA splenectomized between 2000-2018 at Robert Debré University Hospital (Paris, France).

A total of 188 children underwent splenectomy, including 101 (11.9%) from our newborn cohort and 87 referred to our center. Median (Q1-Q3) age at splenectomy was 4.1 yrs (2.5-7.3), with 123 (65.4%) and 65 (34.6%) children splenectomized 77or < 3 years of age, respectively. Median post-splenectomy follow-up was 5.9 yrs (2.7-9.2) yielding 1192.6 patient-years (PY) of observation. Indications for splenectomy were mainly acute splenic sequestration (101 (53.7%)) and hypersplenism 75 (39.9%)). All patients received penicillin prophylaxis, 98.3% received PP23 immunization and 91.9% a median number of 4 (3-4) PCV shots prior splenectomy.

Overall incidence of invasive bacterial infection and thrombo-embolic events were 0.005/PY (no pneumococcal infections) and 0.003/PY, respectively, and did not differ according to age at splenectomy. There was an increased proportion of children with cerebral vasculopathy in children splenectomized < 3 years (0.037/PY vs 0.011/PY, p<0.01). A significantly greater proportion of splenectomized than non-splenectomized children were treated with hydroxycarbamide (77.2% vs 50.1%, p<0.01), suggesting a more severe phenotype in children who present spleen complications.

If indicated, splenectomy should not be delayed in children, provided recommended pneumococcal prophylaxis is available. Spleen complications in childhood may serve as a marker of severity.