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Overwhelming post-splenectomy sepsis with multi-organ dysfunction:  a case report

 
Dr. Mazen Sabah, Dr. Rosemary Phillips and Dr. Jeffrey Phillips*   
 
Department of General Medicine, Princess Alexandra Hospital, Harlow, UK.

* Department of Critical Care, Princess Alexandra Hospital, Harlow, UK


ABSTRACT

Introduction:  Asplenic or patients with splenic dysfunction are at risk of overwhelming infection, most often by encapsulated bacteria.  The risk is greater in the early months and years following splenectomy.

Case presentation:  A case of overwhelming pneumococcal sepsis, occurring 15 years following splenectomy in a 54-year old lady with inactive multiple sclerosis is presented.  The patient rapidly deteriorated after admission to hospital, developing multi-organ dysfunction, disseminated intravenous coagulopathy (DIC), extensive blistering and skin necrosis.  The patient was treated aggressively following the guidelines issued by the Surviving Sepsis Campaign.  She also required prolonged haemodialysis and skin grafting.  The cause, treatment and preventive measures recommended in asplenic and functionally hyposplenic patients are presented. 

Conclusion:  Asplenic patients are at risk of developing infection during their entire life.  Theses patients should be aware of this risk.  These patients should be treated promptly with antibiotics in the event of a febrile episode.  Protective measures should be adequately implemented.  

Keywords: sepsis, splenectomy.


INTRODUCTION

Asplenic patients are known to be at an increased risk of developing serious and potentially fatal sepsis, particularly with encapsulated organisms [1].  The rapidly lethal clinical course demands intense efforts toward prevention, rapid recognition and prompt treatment with broad spectrum antibiotics and supportive measures to reduce morbidity and mortality.

The following report is of a case of pneumococcal sepsis occurring 15 years post splenectomy following a road traffic accident.  It caused considerable morbidity, requiring prolonged hospitalisation.  Appropriate life-long measures could have prevented or ameliorated her illness.

 

CASE PRESENTATION

A 54-year old female presented to the Accident and Emergency Department with a 24-hour history of feeling unwell and sweaty.  She also had rigors and a rash on the face and legs.  In addition, she complained of aches in both legs for five days prior to presentation, for which she had taken a total of five Paracetamol 500mg tablets, over the preceding three days.

Fifteen years previously, she had undergone an emergency splenectomy following a road traffic accident.  The patient was not aware of the need for prophylactic antibiotics and vaccination.  She also had a thirty year history of multiple sclerosis but was in remission and on no medications or immune-modulating therapy, leading a fully independent life. 

Observations on admission were: Temperature: 37.0o C; Heart rate: 120 beats/min, regular; Blood pressure: 90/60 mm Hg; Respiratory rate: 18/min; O2 Saturations were unrecordable on air, rising to 84% on 10 liters of oxygen. Later, a blood gas sample taken from the femoral artery showed O2 Saturation of 96 % on air.

On examination she was fully conscious and orientated.  She had a central cyanosis, non-blanching petechiae over the face and legs and bilateral submandibular lymphadenopathy. 

Blood tests revealed abnormal liver functions, abnormal coagulation screen, hypoglycaemia, severe metabolic acidosis and deranged renal function.  The serum Paracetamol level was in the toxic range.  Blood values are shown in Table 1.


Table 1:  Blood values on admission. 

Table 2:  The recommended vaccinations in asplenia or splenic dysfunction (adapted from [13]).

.

 

Table 1:

Tests

Values

Normal Range

Sodium

136 mmol/l

135 - 145

Potassium

4.4 mmol/l

3.6 - 5.3

Creatinine

192 mmol/l

58 - 96

Urea

11.4 mmol/l

2.5 – 7.5

Glucose

3.2 mmol/l

 

Albumin

36 g/l

36 - 52

Total Bilrubim

23 mmol/l

0 - 17

Alkaline Phosphatase

177 IU/L

30 - 120

ALT

380 IU/L

10 - 31

CRP

296.3 mg/l

0 – 10

Paracetamol

0.22 mmol/l

 

Salicylate

0.39 mmol/l

 

Hb

15.6 g/dl

11.5 – 16.5

WBC

7.2 x10^9/l

4.0 – 11.0

Platelets

116 x10^9/l

150 - 450

MCV

96 fl

76- 96

Neutrophils

6.5 x10^9/l

2.8 – 8.0

PT

23.0sec

11.1 – 13.4

APTT

72.3sec

21.0 – 34.0

INR

2.2

 

D-Dimer

17.57

0 – 0.50


Table 2.

Vaccine type

Frequency & Comment

Pneumococcal

  • The vaccine should be given as a single dose of 23-valent pneumococcal polysaccharide, at least 2 weeks before splenectomy
  • Patients should be given advise about increased risk of pneumococcal infection and given a patient's card and information leaflet
  • Prophylactic antibacterial therapy against pneumococcal infection should not be stopped after immunisation
  • Revaccination every 5 years, as determined by antibody titers

H. influenzae type B

  • Previously immunised adults should be given one additional dose of the vaccine (combined with meningococcal group C conjugate vaccine)
  • For elective splenectomy the vaccine should be given at least 2 weeks before surgery

Meningoccoccal group C

  • Unimmunised adults should be given 2 doses of the vaccine (usually combined with H infuenzae type B) with an interval of 2 months between doses
  • Previously immunised patients should be given 1 additional dose of the vaccine (usually combined with H. influenzae type B vaccine)

Influenza

Recommended yearly to reduce risk of secondary bacterial infection

 

Blood cultures were taken and supportive measures were initiated.  These included IV fluids, oxygen therapy, blood glucose control, administration of broad spectrum antibiotics (Cefatoxime 2g iv), N-Acytel Cysteine infusion and correction of the coagulopathy with fresh frozen plasma and vitamin K. 

The differential diagnoses at this stage included severe sepsis, a vasculitic process, liver dysfunction or possible pulmonary embolus. An urgent Echocardiogram was performed which ruled out haemodynamically significant pulmonary embolus.

Blood cultures grew gram-positive diplococci identified as Streptococcus pneumoniae.

Her conscious level and respiratory function deteriorated rapidly, requiring transfer to the Intensive Care Unit where she received support recommended in the Surviving Sepsis Campaign Guidelines [2], including invasive ventilatory support and the administration of activated protein C.  Haemodialysis was also commenced and continued for 29 days due to poor renal function.  Later on, she developed extensive blistering and large areas of skin necrosis over the lower and upper limbs, face and abdomen with gangrene of the toes (Figure 1). 

Figure 1:  Extensive blistering and necrosis over the face (a) and lower (b, c, d) and upper limbs (e)

 

The leg ulcers became infected and necrotic.  Pseudomonas species was grown from these areas.  After resolution of the critical phase of her illness, which lasted for almost three months, the patient was transferred to the regional burns unit for debridement and skin grafting.  Four months later, the patient is well and satisfied with grafting results.  However more surgical input was required to construct some parts of her nose.  The patient was given the appropriate advice regarding immunisation, antibiotic prophylaxis and medic alert.

DISCUSSION

It is obvious that this patient had severe sepsis.  The abnormal liver functions and raised paracetamol blood level were attributed to the metabolic consequences of severe sepsis contributing to the impaired catabolism of small doses of the drug.  

The clinical consequences of various overwhelming post-splenectomy infections have been well documented.  Patients usually present with fever, brief upper respiratory tract infection that progresses over a few hours to shock, DIC and multiple organ failure.  It has been reported that the calculated incidence of post splenectomy sepsis in unselected patients, including those with immunosuppression, is 0.18 case per 100 per person-years [3].  The risk of sepsis is higher in children [4], patients with compromised humoral immunity and in those who have had an elective splenectomy, especially for haematological malignancies and thalassaemia, as compaired with those who have emergency post-traumatic splenectomy [5].  The risk seems to be is greater in the early months and years following splenectomy [6], but persists throughout life, with 60% occurring 10-30 years after splenectomy [7, 8].  The reported mortality of severe infection in asplenic individuals has varied from 50% to 90% [9, 10]; this appears to be a result of the inclusion in various series of individuals of different age groups, persons with meningitis, and patients with septicaemia but without disseminated intravascular coagulation who appear to have a better prognosis [11].

Although any encapsulated organism can cause infection, Streptococcus pneumoniae is the commonest cause of sepsis in asplenic individuals accounting for up to 90% of such episodes [12].  The mortality rate of pneumococcal sepsis with disseminated intravascular coagulation is extremely high. 

Our patient was unusual because she was one of the few asplenic adults to survive an episode of fulminant infection with disseminated intravascular coagulation and multi-organ failure.

For asplenic patients or those with splenic dysfunction should have vaccinations against pneumococcal, H. influenzae type B, meningoccoccal group C and Influenza.  Different countries may have slight variations in their recommendations.  The recommended vaccines in such patients in the UK are shown in Table 2 [13].

The issue of long-term prophylactic antibiotic therapy remains controversial [14].  However, antibiotic prophylaxis could offer the most benefit in the first 3 years post splenectomy or the first 6 months after the occurrence of a first severe infection [15]. In adults phenoxymethylpenicillin is recommended at a dose of 500 mg every 12 hours for the prevention of pneumococcal infection.  If a cover is also needed for H. influenzae in children amoxicillin can be given instead [16].  Patients who are allergic to penicillin are offered erythromycin.  Patients who travel to endemic areas of malaria should adhere to anti-malarial prophylaxis [17].  Patients who develop an infection despite vaccination and antibacterial prophylaxis should go immediately to hospital for treatment with broad spectrum antibiotics

 

CONCLUSIONS

The above measures will not completely prevent overwhelming infection [18] and asplenic patients should be made aware of their increased susceptibility to infection and the potential dangers of a septic episode following animal and tick bites.  The patients should be advised to carry a card stating their clinical details, immunisation history and useful contact numbers.  

There are a significant number of post-splenectomy patients who do not follow the best practice guidelines [19] and therefore, a high index of suspicion must be maintained for any febrile illness in a functionally hyposplenic or asplenic patient. Should such an illness occur, vigorous investigations and treatment are mandatory.

LIST OF ABBREVIATIONS:  disseminated intravenous coagulopathy, DIC.

CONSENT

A written informed patient consent was obtained for publication of this report and accompanying images.

COMPETING INTERESTS

No competing interests.

AUTHOR’S CONTRIBUATION

Dr. M. Sabah is the first author, was the first to see and manage the patient.

Dr. Rosemary Phillips is a Supervisor (Consultant in Gastroenterology)

Dr. Jeffrey Phillips is a Supervisor (Consultant in Critical Care Medicine)

 


REFERENCES

  • Gopal V, Bisno AL: Fulminant pneumococcal infections in ‘normal’ asplenic hosts. Arch Intern Med 1977, 137:1526-1530
  • Dellinger RP, Carlet JM, Masur H, Gerlach H, Calandra T, Cohen J, Gea-Banacloche J, Keh D, Marshall JC, Parker MM, Ramsay G, Zimmerman JL, Vincent JL, Levy MM: Surviving Sepsis Campaign Guidelines for Management of Severe Sepsis and Septic Shock. Crit Care Med 2004, 32:858-873
  • Schwartz PE, Sterioff S, Mucha P, Melton LJ 3rd, Offord KP: Postsplenectomy sepsis and mortality in adults. JAMA 1982, 12:248:2279-2283
  • Pedersen FK: Postsplenectomy infections in Danish children splenectomized 1969-1978. Acta paediatr Scand 1983, 72:589-595
  • Dickerman JD: Splenectomy and sepsis: a warning. Paediatrics 1979, 63:938-941
  • Waghorn DJ: Prevention of postsplenectomy sepsis [letter]. Lancet 1993, 341:248
  • Holdsworth RJ, Irving AD, Cuschieri A: Postsplenectomy sepsis and its mortality rate: actual versus perceived risks. Br J Surg 1991, 78:1031-1038.
  • Eber SW, Langendörfer CM, Ditzig M, Reinhardt D, Stöhr G, Soldan W, Schröter W, Tchernia G: Frequency of very late fatal sepsis after splenectomy for hereditary spherocytosis: impact of insufficient antibody response to pneumococcal infection. Ann Hematol 1999, 78:524-528.
  • Singer DB: Postsplenctomy sepsis.  In Prespectives in Paediatric Pathology, volume1. Edited by Rosenberg HS and Bolande Bolande RP. Chicago: Year Book Medical; 1973: 285-505.
  • Balfanz JR, Nesbit ME Jr, Jarvis C, Krivit W: Overwhelming sepsis following splenectmy for trauma. J. pediatr 1976, 88:485-460
  • Ramsay LE, Bouskill KC: Fetal pneumococcal meningitis in adults following splenectomy: two case reports and a review of the literature. J R Nav Med Serv 1973, 59:102-114
  • Van Wyck DB: Overwhelming postsplenecetomy infection (OPSI): the clinical syndrome. Lymphology 1983, 16:107-114
  • Immunological products and vaccines. In British National Formulary. 54th edition. BMJ Publishing Group Ltd, London September 2007:634-677
  • Kaplinsky C, Spirer Z: Post-splenectomy antibiotic prophylaxis-unfinished story: to treat or not to treat? Pediatr Blood Cancer 2006, 47:740-741
  • Kyaw MH, Holmes EM, Toolis F, Wayne B, Chalmers J, Jones IG, Campbell H: Evaluation of severe infection and survival after splenectomy. Am J Med 2006, 119: 276.e1-7
  • Infections. In British National Formulary. 54th edition. BMJ Publishing Group Ltd, London September 2007:274-354
  • NHS. Cumbria and Lanncashire Health Protection Unit: Guidelines on the prevention of infection in asplenic patients and patients with dysfunctional spleen. 2002.
  • Evans DI: Fatal post-splenectomy sepsis despite prophylaxis with penicillin and pneumococcal vaccine. Lancet 1984, 1: 1124
  • Spelman D: Prevention of overwhelming sepsis in asplenic patients: could do better. Lancet 2001, 357: 2072

 

Copyright Priory Lodge Education Limited 2008

First Published March 2008


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