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Lemierre Syndrome

Lemierre Syndrome

An 18-year-old woman with no known medical history presented to the ED complaining of fever, chills, and rigors of a week’s duration. The patient appeared very ill with hypotension, tachycardia, tachypnea, and fever. Her initial laboratory studies showed a WBC count of 13,800/µL (normal range 4500 – 11,000/µL); platelets 17,000 (normal range, 150 – 400 x 109/L); creatinine, 1.1 mg/dL (normal range, 0.6 – 1.0 mg/dL); total bilirubin, 2.9 mg/dL (normal range, 0.2 – 1.4 mg/dL). Blood culture was positive for Fusobacterium nechrophorum. Chest films showed a left pleural effusion. A pelvic mass, hepatosplenomegaly, and persistent thrombocytopenia prompted her transfer to the MICU.

On admission to the MICU, the patient was in moderate general and respiratory distress. Her blood pressure was 95/60 mm Hg; respiratory rate, 30 breaths/min; heart rate, 115 beats/min; and temperature, 38.6°C (101.6°F). Oral examination found no lesions, erythema, or exudation or swelling of the posterior pharyngeal mucosa. The tonsils were moderately swollen. Breath sounds were decreased at the left base but there were no rhonchi, wheezes, or crackles. Cardiac examination revealed tachycardia; no murmurs, rubs, or gallops were detected by auscultation. The abdomen was not tender or distended and bowel sounds were present. The palpable mass in the left lower quadrant and significant hepatosplenomegaly noted in the ED were confirmed. Her skin showed no rashes or petechiae, and the neurologic examination was normal. Infectious diseases was consulted. A diagnosis of Lemierre syndrome was proposed and radiologic studies were ordered. CT scans of the chest, abdomen, and pelvis revealed multiple pulmonary nodules and consolidation predominantly in the left lower lobe and also in the right upper and lower lobes.

 

Lemierre's syndrome, pleural effusions, hepato-splenomegaly, hepatosplenomegaly, F necrophorum
Figure 1.


Bilateral pleural effusions, greater on the left than on the right, hepatosplenomegaly, and an ovarian cyst measuring 5 cm were seen on abdominal imaging (Figures 1 and 2). Ultrasonography of the neck showed an acute right-sided deep jugular venous thrombosis. To determine the size of the thrombosis and to rule out compromise of the cavernous sinus, MRI of the brain and a venogram were conducted (Figure 3). Gynecology was consulted for evaluation of the cystic mass in the pelvis. Ultrasonography revealed a hemorrhagic ovarian cyst.

Lemierre's syndrom, F necrophorum
Figure 2.


The patient was allergic to penicillin, so therapy with aztreonam, gentamicin, and clindamycin was started. She was also given crystalloid fluid resuscitation and oxygen through a non-rebreathing mask. In the initial 5 days of antibiotic treatment her, leukocytosis, organomegaly, and thrombocytopenia improved and the pelvic mass became smaller. The patient recovered, with total resolution of the thrombocytopenia.

MRI jugular venous thrombosis, Lemierre's syndrome, F necrophorum
Figure 3.

Discussion
Lemierre syndrome is a series of clinical events that occur after bacterial infection, usually with F necrophorum. The syndrome involves oropharyngeal infection associated with internal jugular vein thrombosis and septic emboli to sites such as lung, joints, and bones.1-6 Although early cases were characterized by infection with F necrophorum, recently other microorganisms have been identified in association with the syndrome.7-9

The majority of isolates of Fusobacterium obtained from infected patients are identified as F necrophorum subspecies necrophorum—the only subspecies able to aggregate human platelets. Streptococcus pyogenes has also been implicated in Lemierre syndrome9 and is known to have platelet activation/aggregation properties similar to those seen with F necrophorum.10

F necrophorum and S pyogenes are found in the normal oral flora. The circumstances under which these bacteria become pathogenic are not well understood. The association of recurrent tonsillitis and the presence of F necrophorum has been demonstrated by polymerase chain reaction analysis. Host factors may play a role in disease production.

The patient we describe here is unique in that Lemierre syndrome and thrombocytopenia in the absence of disseminated intravascular coagulation has not been documented previously. No host factors or other nosocomial influences were found to explain the patient’s thrombocytopenia, which resolved with antimicrobial treatment.

Teaching Points
• Lemierre syndrome is a dangerous treatable infection that should be strongly suspected in any young adult with sore throat and sepsis.
• An early diagnosis is crucial to prevent morbidity and mortality, and targeted radiologic studies are key.
• MRA of the head and neck is one of the most useful diagnostic studies and may quickly determine disease extent and severity.

References

1. Karkos PD, Karkanevatos A, Panagea S, et al. Lemierre’s syndrome: how a sore throat can end in disaster. Eur J Emerg Med. 2004;11:228-230.
2. Georgopoulos S, Korres S, Riga M, et al. Lemierre’s syndrome associated with consumption coagulopathy and acute renal failure: a case report. J Laryngol Otol. 2008;122:527-530.
3. Ravn T, Huniche B, Breum L, Christensen JJ. Lemierre’s syndrome: still an important clinical entity. Scand J Infect Dis. 2006;38:299-301.
4. Riordan T. Human infection with Fusobacterium necrophorum (necrobacillosis), with a focus on Lemierre’s syndrome. Clin Microbiol Rev. 2007;20:622-659.
5. Brown LM, Wallwork B. Lemierre’s—the sinister sore throat. J Laryngol Otol. 2007;121:692-694.
6. Golpe R, Marín B, Alonso M. Lemierre’s syndrome (necrobacillosis). Postgrad Med J. 1999;75:141-144.
7. Hot A, Coppere B, Thiebault A, Ninet J. Lemierre syndrome caused by Leptotrichia buccalis in a neutropenic patient. Int J Infect Dis. 2008;12:339-340.
8. Blumberg D, Brazzola P, Foglia CF, et al. Lemierre syndrome caused by group A streptococci. Pediatr Infect Dis J. 2007;26:661.
9. Anton E. Lemierre syndrome caused by Streptococcus pyogenes in an elderly man. Lancet Infect Dis. 2007;7:233.
10. Forrester LJ, Campbell BJ, Berg JN, Barrett JT. Aggregation of platelets by Fusobacterium necrophorum. J Clin Microbiol. 1985;22:245-249.

 
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