The case of the mysterious vanishing spleen: autosplenectomy complicating pneumococcal sepsis

Abstract

A 57-year-old previously healthy fisherman was admitted in fulminant pneumococcal septic shock, with disseminated intravascular coagulation, requiring aggressive management including bilateral below-knee amputations for ischaemic necrosis. He began to recover and was discharged for rehabilitation, however during his convalescence was found to be hypercalcaemic. No malignancy was found on CT scan, but it was noted that his spleen was absent, replaced by a 4 cm smooth-walled, fluid-filled lesion. This was unexpected as an ultrasound in intensive care 10 weeks previously had demonstrated a normal spleen. Functional hyposplenism was confirmed on a peripheral blood film with evidence of target cells, spherocytes and Howell-Jolly bodies. A diagnosis of autosplenectomy complicating pneumococcal sepsis was therefore made, of which there is just one case previously reported. The patient continues to recover well and was discharged on penicillin prophylaxis after receiving vaccinations for hyposplenism.

Pneumococcal Sepsis as a Cause of Massive Splenic Infarction in Patient with Unknown Celiac Disease

“…In conclusion we would like to emphasise that in patients with a massive bacteremia caused by encapsulated microorganisms and no obvious primary source of infection, it may be useful to investigate and test for a potential disease causing a functional hyposplenism, sometimes discovered in adulthood as for the patient we described in this report [19,20]. In addition, Echo-color Doppler with contrast agent is a useful tool for a diagnosis and follow up of patients with SI.…”

Occurrence of autosplenectomy in a survivor of pneumococcal purpura fulminans

“…To our knowledge, there have been only two reported cases of autosplenectomy after pneumococcal sepsis. 1,2 The speculated mechanism was septic microangiopathy in the spleen after pneumococcal septic shock 1,2 ; however, this case raised a possibility that the patient experienced fulminant pneumococcal infection during anatomical autosplenectomy. Regardless of whether the infection is the cause of autosplenectomy, these patients still require pneumococcal vaccination for vanishing spleen.…”