Background: Severe invasive infections should prompt an immunological investigation. Assessment of splenic function is not typically among the routine immunological work-ups performed in patients with suspected primary immunodeficiency and the presence of Howell Jolley bodies is often relied on as a screening tool.
Methods: we describe 4 cases of functional hyposplenism diagnosed between August 2017 and January 2018, their clinical presentation, infecting organisms, diagnostic methods including the utility of Howell Jolley bodies, and management.
Results: The 4 patients diagnosed with functional hyposplenism were at the ages of 0.8, 1, 1.9 and 32 years. All were administered to Assaf Harofeh medical center with severe sepsis and bacteremia. Blood cultures grew Streptococcus pneumonia serotype 12 in three patients and Hemophilus influenza A in one. A full immunological workup was normal. None of the patients had Howell Jolley bodies on blood smear and all had a spleen demonstrated on abdominal ultrasound. Three patients had constant thrombocytosis (>550K). A spleen scan demonstrated diminished intake in 2 patients and profound minimal intake in the other 2. The 32 year old patient was admitted with severe septic shock and purpura fulminans and required bilateral limb amputation. His medical history reveled prior severe encapsulated bacterial infections in infancy, but no severe bacterial infections later on. He had no immunological inquiry prior to this event.
Conclusions: Functional hyposplenism should be actively investigated in patients with invasive encapsulated bacterial infections whose immunological work-up is otherwise normal. In hyposplenic patients prophylactic antibiotic treatment can prevent serious future infections and resultant devastating outcome.
