Fatal pneumococcal septicemia in a patient with a connective tissue disease

Fulminant pneumococcal sepsis is a rare but life-threatening illness usually occurring in patients with known splenic absence (postsplenectomy) or dysfunction (sickle cell anemia). Several medical illnesses, not typically recognized as being associated with abnormal spleen function, may be complicated by fulminant pneumococcal sepsis. We report a case of fatal pneumococcal sepsis in a patient diagnosed with mixed connective tissue disease who likely had systemic lupus erythematosus and unsuspected splenic fibrosis. Medical illnesses associated with functional asplenia, hematological findings suggesting splenic dysfunction, and confirmatory tests of hyposplenism are discussed.