A 16-year-old boy presents to the emergency department with a 12-hour history of fever and...

Patients with sickle cell anemia often develop functional asplenia due to repeated splenic infarction. The spleen plays a critical role in clearing encapsulated organisms such as Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria meningitidis. Functional asplenia significantly increases the risk of overwhelming sepsis.

Toronto Notes 2023 – Hematology, “Sickle Cell Disease”:

“Functional asplenia is common in sickle cell disease and increases the risk for sepsis due to impaired clearance of encapsulated bacteria.”

MCCQE1 Objectives (Pediatrics > 77-2: Hematology):

“Candidates must recognize that patients with functional asplenia are at increased risk for sepsis and require prompt evaluation for fever.”

Neutrophil dysfunction (B) and immunoglobulin deficiency (C) are not typical features of sickle cell anemia. Chronic anemia (D) and iron overload (E) do not directly increase sepsis risk.

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