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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

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First Published March 2008

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