Splenic sequestration crisis in children with sickle cell disease in the Eastern Region of Saudi Arabia

Background: Splenic sequestration crisis (SSC) is a life-threatening complication of Sickle cell disease (SCD). Studies have shown that patients who carry the African sickle gene haplotypes have more severe SCD than those with the Arab-Indian (AI) haplotype. Our aim was to study the prevalence and the clinical features of SSC among SCD children with both haplotypes living in the same environment in the Eastern region of Saudi Arabia (SA). Patients and Methods: A retrospective cohort study of 340 children with SCD, who visited our hospital from 2010 – 2020 was carried out. The collected clinical data of patients with SSC were compared between two groups of children: Eastern and Southwestern (SW) children. Results: A total of 50 patients with SSC were enrolled in this study, with a sex ratio of 2.1. There were 38 children from the SW region (African-haplotype group), and 12 from the Eastern region (AI-haplotype group). The overall prevalence of SSC was 14.7%, with no significant difference between the two groups, p .60. The African-haplotype group were diagnosed with SCD at a younger age than their peers in the AI-haplotype group [median (IQR)]: 8.5 (6-11) v. 30.5 (24-36) months; p<.001. The median (IQR) steady state HB in the African-haplotype group was significantly lower than that in the AI-haplotype group [8 (7.5-9) v. 9 (8-10) gm/dL]; p<.01. During the 1 ^st SSC there was a significant difference between the African-haplotype group and the AI-haplotype group in the following parameters [median (IQR)]: age: 25(12- 48) v. 72(39-134) months, HB 5.1 (4.4-5.9) v. 6.1 (5.9-6.4) gm/dL, Splenic size on admission 5.5 (3-8) v. 8 (6-9.5) cm and upon discharge 3 (2-5) v. 5 (3.5-5.5) cm below the costal margin. The African haplotype group had a significantly greater number of recurrences than did the AI haplotype group [3.5 (2-6) v. 2 (1.5-2.5)]; p<.05. Conclusion: The overall prevalence of SSC among SCD children in the Eastern region of SA was 14.7% without a significant difference between Eastern SA (AI-haplotype group) and Southwestern SA (African-haplotype group) who lived in the same environment. SW Children experienced more severe SSC, suggesting that genetic rather than environmental factors are responsible for the severity of these episodes.