The prevalence and locations of skeletal lesions in children with sickle cell disease (SCD) may be underestimated, with a study offering a basis for individualized treatment of these patients.

The study was published in the European Journal of Haematology.

“Early onset injuries may be asymptomatic, leading to a diagnostic delay; early therapeutic interventions may significantly improve prognosis and quality of life,” the authors wrote. “The current study aimed to investigate the prevalence of unknown bone necrosis in pediatric age; furthermore, we evaluated retrospectively factors potentially associated with a higher risk of lesions.”

The research team conducted an observational, cross-sectional study on 42 pediatric patients (age 7-17) with SCD genotype HbSS, HbS/β0, HbS/β+, and HbSC at a single center in Italy. They were screened by magnetic resonance imaging for avascular necrosis in the spine, upper arms, and femurs, thereby broadening the assessment conducted in most studies to date, which have only explored the hip and femoral head.

Read more about SCD complications

The primary outcome measures were the prevalence and location of bone lesions, and secondary outcomes included differences in sociodemographic characteristics and risk factors for bone lesions and vaso-occlusive crises among the studied patients.

The results revealed a higher than expected prevalence of occult bone lesions, particularly in the vertebrae and humeri. Hip necrosis prevalence was similar to that published in other studies. The results also showed that a single vaso-occlusive crisis can result in a bone infarct, and that patients could still be asymptomatic.

Finally, the explorations did not find any particular sociodemographic, genetic, or biochemical variables that predicted a greater risk of bone lesions in the patient cohort. Therefore, the authors strongly recommend early and broad assessment of bone damage in children with SCD to individualize their treatment and follow-up and to prevent complications.

Reference

Voi V, Turrini S, Mattavelli M, et al. Occult ischemic bone lesions in children with sickle cell disease: a study of prevalence. Eur J Haematol. Published online November 11, 2022. doi:10.1111/ejh.13898