Prevalence and predictive factors of splenic sequestration crisis among 423 pediatric patients with sickle cell disease in Tunisia

Blood Cells Mol Dis. 2020 Feb;80:102374. doi: 10.1016/j.bcmd.2019.102374. Epub 2019 Oct 18.

Abstract

This study was aimed to identify the predictors of splenic sequestration crisis (SSC) among pediatric patients with sickle cell disease (SCD). This prognosis study was carried out in the pediatric immuno-hematology unit, over 20 years (1998 to 2017), enrolling patients with SCD. The cox model was used in multivariate analysis. Among 423 patients with SCD (240 S/S phenotype, 128 S/B0, 30 S/B+, 14 S/O arab and 11 S/C), 150(35.4%) had at least one episode of SSC. The average age of patients at the first episode was 48.3 months ± 32.4(2-168). Recurrence of SSC was observed in 117 patients (78%). Spleen size ≥3 cm at baseline was the strongest predictor of SSC occurrence (HR = 7.27, CI: 4.01-13.20, p = 0.05) and recurrence (HR = 6.37, CI: 1,46-27.83, p = 0.01). Pallor revealing the disease, age at onset of symptoms <24 months and reticulocytosis ≥300,000/mm3 increased the risk of SSC. Pain crisis revealing the disease as well as neutrophilia was associated with a lower risk of SSC. In conclusion, this study confirmed the high prevalence of SSC in SCD and the high frequency of recurrence after a first episode. The SSC occurrence and recurrence were intimately linked to the presence of splenomegaly, chronic pallor revealing the disease as well as reticulocytosis.

Keywords: Acute splenic sequestration; Children; Sickle cell disease; Spleen.

MeSH terms

  • Acute Disease
  • Anemia, Sickle Cell / complications*
  • Anemia, Sickle Cell / diagnosis
  • Anemia, Sickle Cell / epidemiology*
  • Biomarkers
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Incidence
  • Infant
  • Male
  • Phenotype
  • Prevalence
  • Prognosis
  • Public Health Surveillance
  • Risk
  • Splenic Diseases / diagnosis
  • Splenic Diseases / epidemiology*
  • Splenic Diseases / etiology*
  • Splenomegaly
  • Tunisia / epidemiology

Substances

  • Biomarkers