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Updated: Sep 11, 2025
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Fever in Sickle Cell Disease: Thinking Beyond Bacteremia
Sweta Dubey1,2, Shubhangi Tuli1,2, Sana Usmani2
1Department of Pediatrics, SUNY Downstate Health Sciences University, Brooklyn, New York.
Insights
Fever in pediatric sickle cell disease (SCD) often suggests infection. However, this case highlights that prolonged fever in SCD may stem from noninfectious causes, requiring thorough investigation beyond typical antibiotic treatment.
Area of Science:
- Hematology
- Pediatrics
- Immunology
Background:
- Sickle cell disease (SCD) presents complex pathophysiology and diverse symptoms.
- Fever is a common reason for emergency department visits in pediatric SCD patients.
- Immunocompromised status in SCD due to hyposplenism elevates infection risk.
Purpose of the Study:
- To present a case of prolonged fever in an adolescent with SCD.
- To explore noninfectious etiologies of fever in SCD patients.
- To emphasize the importance of considering noninfectious causes in persistent fever.
Main Methods:
- Case report of an adolescent female with SCD experiencing prolonged fever.
- Initial treatment focused on presumed infection with antibiotics.
- Subsequent investigations to identify noninfectious causes of fever.
Main Results:
- The patient presented with prolonged fever and a complicated clinical course.
- Antibiotic therapy for presumed infection was ineffective.
- Further investigations revealed noninfectious etiologies for the fever.
Conclusions:
- Fever in sickle cell disease necessitates careful evaluation for both infectious and noninfectious causes.
- Prolonged or unresponsive fever warrants a broader differential diagnosis.
- Noninfectious conditions should be considered in the management of febrile SCD patients.
Abstract:
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.
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