Fever in Sickle Cell Disease: Thinking Beyond Bacteremia.

Sickle cell disease (SCD) is a complex pathophysiology with a wide range of symptoms. More than one-third of pediatric SCD emergency department encounters are due to fever. Patients with SCD are immunocompromised because of hyposplenism. Therefore, infection is a serious concern in any febrile patient with SCD. Although bacteremia is seen in only 1.1% of febrile patients with SCD, it remains the leading cause of death globally. However, several noninfectious causes of fever can be seen in SCD, which should be considered in appropriate settings. We present a case of an adolescent female patient with SCD who had an unusually prolonged fever and a complicated disease course that was initially attributed to infection but failed to resolve by appropriate antibiotics. Further investigations identified noninfectious etiologies of fever in the patient.