Acute Splenic Sequestration in Sickle Cell Disease (SCD): Still a Life Threatening Complication

Background: Acute splenic sequestration (ASS) is a life threatening event in SCD during early childhood. Subsequent related mortality has been greatly reduced by both parental education and appropriate management. Moreover SCD screening in neonatal period has allowed early detection contributing to the decrease in fatal attacks.

Objectives: Our aim was to update splenic sequestration epidemiology in a French cohort followed after neonatal screening.

Methods: we reviewed the medical files of SCD children born between 2000–2007 followed since birth in 5 pediatric centres. We included all SCD children diagnosed with acute splenic sequestration, defined as an acutely enlarging spleen with a fall of hemoglobin (Hb) concentration of at least 2g/dL.

Results: A total of 266 episodes of ASS occurred in120 children. There were 69 boys and 51 girls. Genotypes were as follows: 9 S beta° thalassemia, 2 SD-Punjab, 4 SC, 105 SS. In the SS subgroup, median age at first episode was 16 months [1–83] and 34,8% of these first episodes occurred before 12 months of age. Associated symptoms were found in 37 cases (isolated fever n=20, vaso occlusive crisis n=5, identified viral or bacterial infection n= 12) and did not influence the rate of recurrence. Mean Hb level during first crisis was 5.2g/dL [3.3–7]. Mean age at first episode for those who experienced only one episode (n=43) versus more than one episode (n=62) was statistically different (18 months (+/−10) versus 28 (+/−22); p=0.002, Student test). Median interval between first and second episode was 3.5 months [1–31]. 31 patients had 3 or more episodes with a mean interval between second and third interval of 2 months [1–17]. There was no significant difference in the mean age at first episode in children who experienced 2, 3 or more episodes. There was only one fatal case due to acute anemia which occurred in a girl at first recurrence (Hb:2,2g/dL). After the second attack 14(22.5%) patients were splenectomized, 26 patients (41.9%) were started on a transfusion program (followed in 13 cases by splenectomy) and 21 were followed up. Splenectomy was performed at a median age of 4.5years.

Conclusion: These results show that ASS remains a major concern in the management of SCD children. We show a high rate of recurrence and young age at first episode favours this recurrence threat. This study further confirms the effectiveness of early diagnosis and parental education in the decrease of mortality. Prospective studies on spleen dysfunction and additional research on predictive factors are warranted.