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

BACKGROUND

Acute splenic sequestration crisis (ASSC) is a potentially life-threatening complication of sickle cell disease (SCD). Previous studies have shown that patients who carry the African sickle gene haplotypes have more severe SCD than those with the Asian haplotype. In Saudi Arabia (SA), people living in the eastern region are known to carry the Asian haplotype, whereas patients in the southwestern (SW) region are more likely to carry the African haplotype. The aim of this study was to determine the prevalence and clinical features of ASSC among children with SCD who originated from both regions but were living in the same environment in the eastern region of that country.

METHODS

This retrospective cohort study included 340 children with SCD who visited our hospital from 2010 to 2020. The collected clinical data of patients with ASSC were compared between two groups of patients: eastern and SW children.

RESULTS

Over the study period, out of 340 children with SCD, 50 patients had ASSC, with a male/female ratio of 2.1:1. There were 38 children from the SW region and 12 from the eastern region. The overall prevalence of ASSC was 14.7%, with no significant difference between the two groups (p = .56). The SW group was diagnosed with SCD at a younger age than their eastern peers [median (IQR)]: 8.5 (6-11) vs. 30.5 (24-36) months; p < .001. The median (IQR) steady state hemoglobin (HB) level in the SW group was significantly lower than that in the eastern group [8 (7.5-9) vs. 9 (8-10) gm/dL]; p = .001]. During the first instance of episode of ASSC, there was a significant difference between the SW group and the eastern group in the following parameters [median (IQR)]: age [25 (12-48) v. 72 (39-134) months, p = .001], HB [5.1 (4.4-5.9) v. 6.1 (5.9-6.4) gm/dL, p = .01], splenic size on admission [5.5 (3-8) v. 8 (6-9.5) cm, p = .01], and splenic size upon discharge [3 (2-5) v. 5 (3.5-5.5) cm below the costal margin, p = .02]. The SW group had a significantly greater number of recurrences than did the eastern group [3.5 (2-6) v. 2 (1.5-2.5); p = .03].

CONCLUSIONS

The overall prevalence of ASSC among SCD children in the eastern region of SA was 14.7%, and there was no significant difference between the eastern and SW groups. Despite living in the same environment in the eastern region, children from southwestern SA experienced more severe ASSC manifestations than their eastern peers.

TRIAL REGISTRATION: Not.