Prognostic significance of early acute splenic sequestration in children with severe sickle cell genotypes: A comprehensive longitudinal neonatal cohort study

Abstract

Acute splenic sequestration crisis (ASSC) is one of the earliest acute clinical manifestations of sickle cell anemia (SCA), with a median age at first episode of 1.8 years [range: 0.4-12.9] as reported for our recently published regional longitudinal newborn cohort, beginning with the introduction of newborn screening (1986) and ending just before the introduction of preventive intensification with hydroxyurea (HU) in 2015. 1 Early predictive biomarkers have been identified for ASSC, but little is known about the impact of early ASSC on disease severity. 2 Unlike early dactylitis, early ASSC was not found to be associated with an increase in the risk of adverse outcomes, including death, stroke, frequent vaso-occlusive crisis (VOC), and recurrent acute chest syndrome (ACS), in a cohort of newborns with SCA. 3