Age: 67.0
Sex: F
Vaccine: PPV
Symptoms: Shock, Vomiting, White blood cell count increased
Clinical narrative: Information has been received from a published article that an overwhelming postsplenectomy infection (OPSI) is a low-incidence entity with a high mortality rate despite aggressive therapy. Although initial symptoms may be mild and nonspecific, it can progress rapidly to Waterhouse-Friderichsen syndrome with full blown septic shock and disseminated intravascular coagulation (DIC). Overwhelming postsplenectomy infection is known to occur at any time after splenectomy, even in patients who have received pneumococcal immunization and/or chemoprophylaxis. Although the term OPSI gives the impression of a "postsurgical" complication, it has been seen in association with conditions predisposing to functional hyposplenism and in children with congenital asplenia. The physicians reported there had been no previously reported cases of OPSI in a pneumococcal vaccinated adult with congenital asplenia. A 67 year old woman with congenital asplenia presented to the emergency department complaining of chills, fever, severe low back pain, and vomiting of 24 hours of duration. She had a recent history of a treated urinary tract infection and a remote history of sepsis and pneumonia, for which she was hospitalized and treated in other institutions. The patient also had a diagnosis of lung fibrosis of uncertain etiology and was a chronic alcohol user. The patient had received pneumococcal vaccination 1 year prior. On physical examination, she was found to be alert and in no acute distress. Her temperature was 38 degrees C orally; blood pressure, 100/42 mmHg; heart rate, 113/min; respiratory rate, 26/min; and oxygen saturation, 95% on room air. She had mild tenderness to palpation in the right lower abdominal quadrant and positive bowel sounds. Her initial blood workup showed an elevated white blood cell count of 15.5 x 10^3/mm^3 with 94% neutrophils. A chest x-ray revealed right lung haziness. A computer tomographic scan of the abdomen and pelvis documented no acute intraabdominal process. Blood and urine samples were obtained for cultures. Within a few hours of presentation, the patient developed a diffuse purpuric rash with severe hypotension. Cefepime and vancomycin were started. Additional laboratory tests revealed disseminated intravascular coagulation (DIC) and metabolic acidosis. Cortisol levels were within normal limits, although lower than expected considering the patient's condition. Despite administration of vasopressors agents and intravenous fluids, she developed shock and died in less than 24 hours after presentation. At autopsy a diffuse purpuric rash was noted. There was massive bilateral adrenocortical hemorrhagic necrosis. A recent subdural hemorrhage and scattered focal hemorrhages in the heart and gastrointestinal tract were present. The right lung was extensively fibrotic. Interstitial fibrosis and bronchiectasis were seen bilaterally. There were focci of organizing pneumonia in the right upper lobe and a focus of acute bronchitis in the right middle lobe. The liver showed severe steatosis and findings consistent with chronic alcoholic liver disease. Arterionephrosclerosis was seen in both kidneys. The bone marrow was hypercellular with a few paratrabecular aggregates of monotonous appearing lymphocytes. Asplenia was the only congenital abnormality. Premortem blood cultures reported after death grew Streptococcus pneumoniae sensitive to penicillin and cefotaxime. The urine culture had negative results. Although the patient provided a history of congenital asplenia, the absence of alarming signs at presentation, in addition to an up-to-date pneumococcal vaccination and noncritical initial physical examination and laboratory studies, gave the false impression of a relatively stable patient who could be worked up for a precise diagnosis and subsequent treatment. Prior pneumococcal vaccination should not be reassuring as seen in this patient who in vivo blood cultures grew Streptococcus pneumoniae. The protective role of pneumococcal 23-valent vaccine for adults aged greater than 65 years remains controversial because some older patient do not develop an immune response to the most prevalent serotypes causing invasive disease. In addition, antibody concentrations and response may be lower in elderly patients with comorbid illnesses such as alcoholic cirrhosis, chronic obstructive pulmonary disease, and lymphoproliferative disorders. The 67 year old patient had comorbid illnesses including chronic alcoholic liver disease and severe lung fibrosis. Because OPSI Is a virulent entity with a 50% to 80% case fatality ensuing in less than 48 hours, diagnostic workup should never delay the initiation of empiric antibiotic therapy. As seen in this case, when the initial clinical presentation is mild or nonspecific, physicians may have a false sense of security and attempt to reach a precise diagnosis instead of intervening early with empiric antibiotics. Consequently, this may result in death, even when the delay is only of a few hours. Overwhelming postsplenectomy infection can occur at any time after splenectomy and as demonstrated by this patient should also be considered in adults with congenital asplenia. Additional information is expected. A copy of the published article is attached as further documentation of the patient's experience. 9/3/09 Per FDA  COD=Pneumococcal Sepsis.