Clinical Spectrum of Serious Bacterial Infections Among Splenectomized Patients with Hemoglobinopathies in Israel: a 37-year Follow-up Study

Purpose: Patients with hemoglobinopathies who undergo splenectomy are at risk for invasive infections. The aim of this investigation was to present the clinical spectrum of infections in splenectomized patients.

Methods: The study cohort comprised 54 splenectomized patients with beta-thalassemia (β-thalassemic) and sickle cell disease. The incidence of serious invasive bacterial infections was recorded. All patients received pneumococcal vaccine and all received oral prophylactic penicillin.

Results: A total of 22 episodes of serious bacterial infections were identified in 19 patients among the study cohort of 54 splenectomized patients (35%). The clinical spectrum included sepsis (10 patients), bacteremia (8), liver abscess (1), forearm abscess (1), and urinary tract infection (2). The most frequent pathogens were Escherichia coli (8 cases), Steptococcus pneumoniae (5), and Campylobacter (2). 22 patients with β thalassemia died during the study period: 6 due to bacterial infection and 18 due to cardiomyopathy. The time elapsed between splenectomy and S. pneumoniae infection was significantly shorter than that between splenectomy and infections caused by other pathogens (18 ± 14 vs. 115 ± 93 months, respectively; p = 0.035).

Conclusions: Splenectomized patients with β thalassemia and sickle cell disease are predisposed to severe infections, with the majority of these infections being caused by Gram-negative microorganisms. The attending physician(s) should take these findings into consideration when deciding upon an empiric antibiotic treatment for splenectomized patients who present with fever or sepsis.