Hemoglobin S, the Pneumococcus, and the Spleen

Hemoglobin S (Hb S) and selected bacteria are among medicine's more enigmatic bedfellows. The clinical importance of this association is underscored by the recognition of infection as the major presenting manifestation of sickle cell anemia in early childhood, the most common complication requiring hospitalization and the most common cause of death at all ages. The capacity for Salmonella to haunt patients with sickle cell anemia is widely acknowledged. More recently the vulnerability of children with sickle cell anemia to life-threatening pneumococcal infections has become apparent.¹ That the pneumococcus is the most common cause of bacteremia and meningitis in patients with sickle cell anemia is amply confirmed.

The report by Seeler and associates which appears in this issue (page 8) together with the recently published analysis of bacterial infections in a large group of subjects with sickle cell anemia² permit further characterization of the pneumococcal complications of this hemoglobinopathy.