Safety and Long-Term Effects of Splenectomy At the Age of Two for Acute Splenic Sequestration in Sickle Cell Disease: A Retrospective Review

Despite improved awareness and education about acute splenic sequestration (ASS) in sickle cell disease (SCD), such episodes remain potentially fatal and treatment options are limited. We report our local experience in children splenectomized at the age of two by evaluating long term complications related to sepsis, thrombosis and iron overload.

Retrospective charts review of children with SCD and a history of ASS who underwent total splenectomy between 1996 and 2012 at Ste-Justine Hospital in Montreal, Canada. T-test and Fisher's exact test were used for statistical analysis.

20 patients were included in the study. Median age at splenectomy was 2 years old (range: 2–9 y.o). Median post-splenectomy follow-up was 4 years (range: 1month–16 years). 7 patients (35%) had limited fever in the postoperative setting, including one requiring antibiotics for pneumonia. Median length of stay was 4 days (range: 2–8 days). Three patients were hospitalised for documented sepsis at extended follow-up: two for salmonella at 1 and 12 years post splenectomy, while the third had hemophilus influenzae sepsis five months post-op but also within two months of a sibling cord blood transplantation. Platelet count increased within one month of surgery, and remained elevated at 6 and 12 months post splenectomy (p<0.001 at 1, 6 and 12 months compared to pre-surgery). Deep venous thrombosis was documented in one patient 4 years post splenectomy during hospitalisation for pain crisis. The thrombosis resolved with a three months treatment with enoxaparin. Thrombophilia work-up was negative. 75% of patients were on a transfusion program prior to splenectomy for a median duration of 13 months (11–20 months). Preliminary evaluation of iron overload in seven patients revealed a decrease in ferritinemia in all but two. One patient remained with severe iron overload and still requires oral chelation therapy. HbSS and HbS-Bthal splenectomized patients were more likely to be placed on hydroxyurea (HU) therapy than non-splenectomized patients with an odds ratio of 3 (11/20 vs 39/136 of our local population, p=0.023; 95% CI 1.17–7.9). 2 patients are on a regular transfusion program for elevated transcranial Doppler velocities. One patient underwent match sibling allogeneic transplantation.

Our data suggest that performing total splenectomy at a very young age is a well-tolerated and efficacious treatment modality for children with severe and/or recurrent ASS. Performing splenectomy at a younger age may avoid unnecessarily extended transfusion programs and contribute to lessen the degree of iron overload. We consider ASS as a marker of SCD severity, as illustrated by the high rate of HU use in splenectomized patients. Whether splenectomy leads to biological modification contributing to increased SCD severity should be further studied. Concerns over possible increased thrombotic risk should also be further evaluated.

No relevant conflicts of interest to declare.