Da

Very Ill
Immune System
Diagnosis and reasoning

This patient has presented with signs and symptoms suggestive of acute infection, in association with altered mentation,
tachypnea, tachycardia, and hypotension. Furthermore, his complete blood count is significant for a marked neutrophilia.
The above findings immediately raise the specter of sepsis and septic shock. Urgent resuscitation and stabilization, including
intravenous (IV) fluid therapy, is a must, along with close observation in a critical care setting.
Evaluation of baseline function is also important. In this regard, arterial blood gases reveal metabolic acidosis, an ominous
finding. However, the coagulation profile is normal, as are renal and liver profiles, suggesting that disseminated intravascular
coagulation (DIC) and multi-organ failure are yet to set in.
Following stabilization, an urgent search should be made for the source of sepsis. Unfortunately, examination fails to throw up
any localizing signs of infection, while the negative chest x-rays and urinalysis make pneumonia and urinary tract infection
further unlikely.
That said, the history does provide a very important clue: namely, the splenectomy three years ago.
Asplenic patients are highly susceptible to infection by encapsulated bacteria such as S. pneumoniae, N. meningiditis, and H.
influenzae. These infections may progress into fulminant sepsis within a matter of hours, with this being termed overwhelming
post-splenectomy infection (OPSI).
The uncertainty about his vaccination status adds more fuel to this fear.
Where OPSI is likely, aggressive empirical antibiotic therapy is essential, with the combination of intravenous (IV) vancomycin
and ce riaxone being preferred. Blood, urine, and CSF cultures should be obtained beforehand, and the drug regimen adjusted
appropriately once culture results are available.
Note that noradrenaline therapy is not indicated immediately, but may be required if fluid replacement fails to stabilize the
patient.
Discussion
Overwhelming post-splenectomy infection (OPSI) is a progressive and fulminant bacteremia a ecting hyposplenic or asplenic
patients. It is a medical emergency, with a mortality rate of 38% to 70%, even with optimal treatment.
The incidence of OPSI is estimated to be 2-5 per 1,000 asplenic patients per year. Overall, each asplenic patient will have a 5%
lifetime risk of developing the condition.
To understand the underlying pathophysiology, one must recall that the spleen is the key site of production of antigen-specific
IgM antibodies, which opsonize encapsulated bacteria. Those opsonized bacteria are then removed by macrophages present in
the splenic circulation.
Thus, in hyposplenic or asplenic patients, humoral immunity is greatly a ected, rendering them highly susceptible to infection
by encapsulated bacteria. Such infections can then proliferate rapidly and cause a fulminant sepsis.
S. pneumoniae accounts for over half of all cases of OPSI; N. meningitidis and H. influenzae are also commonly encountered. C.
canimorsus is encountered occasionally, following dog bites.
Patients are at the highest risk of developing OPSI within the first two years following splenectomy, although cases have been
reported as long as 20 years a erward.
Other known risk factors include age below 16 or above 50 years, co-existing hematological disorders or malignancies, ongoing
immunosuppression, previous invasive pneumococcal disease, and poor or no response to vaccination against the common
pathogens.
Initially, these patients experience mild and nonspecific symptoms such as fatigue, malaise, abdominal pain, or nausea.
Subsequently, fever, headache, arthralgias, myalgias, vomiting, or rashes may occur.
If not detected and treated promptly, septic shock and disseminated intravascular coagulation (DIC) will occur, followed by
multi-organ failure. Very importantly, the time from the onset of symptoms to death may be as short as 24 to 48 hours.
Where OPSI is suspected, several urgent steps need to be taken. Close supportive care is essential, including fluid resuscitation to
maintain tissue perfusion; vasopressors or inotropes may also be required. Adequate oxygenation is also important, with
intubation needed if respiratory distress occurs.
Aggressive intravenous empirical antibiotic therapy is also a must, with the combination of vancomycin and ce riaxone being
preferred. The former has excellent gram-positive coverage, including against penicillin-resistant S. pneumoniae, while the latter
adds double coverage of gram-positive organisms, while further including gram-negative coverage against N. meningitidis and H.
influenzae.
Extensive and comprehensive diagnostic testing should proceed in parallel. Complete blood counts o en reveal a leukocytosis,
although leukopenia is also possible. Coagulation profiles may reveal features suggestive of DIC. Where there is multi-organ
failure, liver and renal profiles may be abnormal.
A complete septic screen is also important, including blood and urine, and cerebrospinal fluid (CSF) cultures, as well as chest x-
rays. Note, however, that lumbar punctures are contraindicated in patients with DIC.
Given the high mortality and morbidity of OPSI, prevention is far preferable. Following splenectomy, patients should be
educated about the condition and instructed to seek medical help promptly should they notice any symptoms of infection.
Furthermore, vaccination against pneumococcus, meningococcus, and H. influenzae is essential. These individuals should also
receive annual vaccinations against influenza, to avoid secondary bacterial infection.
The use of prophylactic antibiotics is controversial, given the known negatives of development of bacterial resistance, non-
compliance and a false sense of security. However, this may yet be considered in patients at continued high risk of pneumococcal
infection.
Patients may also be provided with a supply of appropriate antibiotics for emergency use, to be self-administered in case of
infection.
Take home messages

1. Overwhelming post-splenectomy infection (OPSI) is a rapidly progressive, fulminant bacteremia which occurs in hyposplenic
or asplenic patients.
2. A ected individuals initially present with mild and nonspecific symptoms, but then rapidly progress to septic shock and multi-
organ failure if not treated promptly.
3. Early empirical antibiotic therapy is key to the management, with the combination of intravenous (IV) vancomycin and
ce riaxone preferred currently.
4. Vaccination against pneumococcus, meningococcus and H. influenzae is key to the prevention of OPSI.
References
1. SINWAR PD. Overwhelming post splenectomy infection syndrome - review study. Int J Surg [online] 2014 Dec, 12(12):1314-6
[viewed 23 February 2017] Available from: http://www.ncbi.nlm.nih.gov/pubmed/25463041
2. DAVIES JM, LEWIS MP, WIMPERIS J, RAFI I, LADHANI S, BOLTON-MAGGS PH, BRITISH COMMITTEE FOR STANDARDS IN
HAEMATOLOGY.. Review of guidelines for the prevention and treatment of infection in patients with an absent or
dysfunctional spleen: prepared on behalf of the British Committee for Standards in Haematology by a working party of the
Haemato-Oncology task force. Br J Haematol [online] 2011 Nov, 155(3):308-17 [viewed 25 December 2017] Available from:
http://www.ncbi.nlm.nih.gov/pubmed/21988145
3. THEILACKER C, LUDEWIG K, SERR A, SCHIMPF J, HELD J, BöGELEIN M, BAHR V, RUSCH S, POHL A, KOGELMANN K, FRIESEKE
S, BOGDANSKI R, BRUNKHORST FM, KERN WV. Overwhelming Postsplenectomy Infection: A Prospective Multicenter Cohort
Study. Clin Infect Dis [online] 2016 Apr 1, 62(7):871-878 [viewed 25 December 2017] Available from:
http://www.ncbi.nlm.nih.gov/pubmed/26703862
4. RUBIN LG, SCHAFFNER W. Clinical practice. Care of the asplenic patient. N Engl J Med [online] 2014 Jul 24, 371(4):349-56
[viewed 25 December 2017] Available from: http://www.ncbi.nlm.nih.gov/pubmed/25054718
5. DI SABATINO ANTONIO, CARSETTI RITA, CORAZZA GINO ROBERTO. Post-splenectomy and hyposplenic states. The Lancet
[online] 2011 July, 378(9785):86-97 [viewed 25 December 2017] Available from: doi:10.1016/S0140-6736(10)61493-6
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