A 42-year-old man developed sudden onset of fever, chills and generalized weakness one morning. This was preceded by several days of cough and sore throat. He contacted a provincial phone service staffed by registered nurses, who advised him on the treatment of "flu-like" illnesses. About six hours later, he presented to a regional urgent care centre.As a child, the patient had a splenectomy for an unclear hematologic diagnosis. At age 26, he underwent allogenic liver transplantation for primary sclerosing cholangitis. Most recently, he had been treated with cyclosporine (50 mg orally twice daily) for chronic immunosuppression. He had regular follow-up and was considered well. He had received vaccination against infections postsplenectomy, including a booster pneumococcal vaccination (type unknown) within the two years before his current presentation. Peripheral blood smears taken before admission showed Howell-Jolly bodies consistent with asplenia.At the time the patient was assessed in the urgent care centre, he was syncopal, with a blood pressure of 64/49 mm Hg, sinus tachycardia at 140 beats/minute, and a respiratory rate of 30 breaths/ minute. He was febrile at 39°C. Because he was critically ill, he was transferred on an emergent basis to our tertiary care hospital.In the emergency department, intravenous resuscitation was started, using large volumes of isotonic crystalloid; broadspectrum antibiotics were given. The patient required intravenous vasopressors for blood pressure support. A chest radiograph showed central peribronchial thickening and increased interstitial markings in both lower lobes. His serum lactate was elevated at 8.4 (normal ≤ 2.0) mmol/L, and his serum pH was 6.95 (7.36-7.44). His white blood cell count was 3.2 (normal range 4.0-11.0) × 10 9 /L, with circulating premature leukocytes including 16% bands. He had multiple organ failure, including shock, respiratory failure, disseminated intravascular coagulation, hepatocellular dysfunction and renal failure.The patient died about seven hours after presentation. Blood and sputum cultures grew Streptococcus pneumoniae (blood culture incubation positive at 5.4 hours) sensitive to penicillin. The patient's next of kin consented to postmortem examination, which confirmed the cause of death as septic shock from overwhelming postsplenectomy infection. Subsequent S. pneumoniae serotyping confirmed serotype 15C.
DiscussionThis case illustrates the risk of fulminant sepsis and death in patients who have undergone prior splenectomy. Asplenia (surgical splenectomy) or hyposplenism secondary to an underlying medical condition is common. An estimated prevalence of asplenia in an Eastern Canadian cohort was 1 per 1000 persons. 1 In this cohort, 48.6% of patients had a splenectomy for a preexisting hematologic diagnosis, such as immune thrombocytopenic purpura. Splenic rupture from trauma has been a common indication for splenectomy; however, with the advent of therapies such as endovascular embolization, the frequency of splenectomy in trauma is decreasi...