Infection in sickle cell disease

Bacterial infections are responsible for a majority of morbidity and mortality in children with SCD. (1,2)

The following factors are thought to increase the susceptibility of affected children:

• splenic hypofunction
• defects in complement activation
• deficiencies of micronutrient
• tissue ischaemia

The common pathogens include:

• Pneumococcus
  • Streptococcus pneumoniae sepsis is the commonest cause of death in children with SCD
  • vaccination and prophylactic penicillin can be used for prevention (2)
• Haemophilus
  • immunisation has reduced the risk of infection significantly
• Salmonella

Reference

1. Rees DC, Williams TN, Gladwin MT. Sickle-cell disease Lancet. 2010 11;376(9757):2018-31
2. Bain BJ, Daniel Y, Henthorn J, et al. Significant haemoglobinopathies: a guideline for screening and diagnosis: a British Society for Haematology guideline. Br J Haematol. 2023 Jun;201(6):1047-65.