Lemierre's Syndrome (also known as postanginal septicemia and human necrobacillosis) was first described by French physician and microbiology professor Andre Lemierre in 1936. He classified this syndrome as an "anaerobic postangial sepsis" caused primarily by Bacillus funduliformis (known today as Fusobacterium necrophorum), a gram-negative anaerobe (organism on SBA, right). This organism is responsible for 90% of Lemierre's syndrome cases and 21% of recurrent sore throats.
Before the introduction of antibiotics, Lemierre's syndrome had a mortality rate around 90%. Currently, the mortality rate of correctly-diagnosed cases is around 5%. Fewer than 100 cases have been described since 1974, and the condition has a incidence rate of 3.6 cases per 1 million. This syndrome is characterized by a recent history of oropharyngitis with persistent fevers, followed by septic thrombophlebitis (swelling of a vein caused by a blood clot) of the internal jugular vein as well as dissemination of the infection to multiple sites. Lemierre's syndrome should be suspected in patients who present with pharyngitis and high-grade fevers (39-41C) plus thrombophlebitis or sepsis.
Case Report
A previously healthy 44 year old female presented with a 3 day history of worsening sore throat predominantly on the left side with fever, pain while swallowing, and vomiting. Upon examination, she was febrile, hypotensive, and tachycardic. Her left tonsil was displaced, and her lymph nodes were swollen and tender. Laboratory testing showed her WBC to be elevated with a decreased platelet count, as well as decreased liver and kidney function. A CT scan (right) showed a mass in the left peritonsillar region (arrow). Drainage of the mass was delayed due to the patient's unstable condition. The patient deteriorated rapidly, developing acute renal failure, encephalopathy, respiratory failure, and bilateral pleural effusions. She was placed on mechanical ventilation and dialysis. Blood cultures grew F. necrophorum and the patient's antibiotics were adjusted to piperacillin-tazobactam and metronidazole. The patient was extubated on her ninth day of admission, and discharged on the thirteenth.
References
SBA picture: http://pictures.life.ku.dk/atlas/microatlas/veterinary/bacteria/Fusobacterium_necrophorum_A/fusobacteriumnecrophoruma.jpg
Case Study: : Arora T, Wright D (2014) Lemierre’s Syndrome in an Adult: A Case Review. Otolaryngology 4: 167. doi:10.4172/2161-119X.1000167
I find that diseases such as these that are rare are scary. Lemierre’s Syndrome also appears to have been discovered relatively recently, or described recently, at only the year 1936. To me at least, something discovered at around this time frame does not seem to have been around that long. The symptoms do not sound pleasant, especially having a “displaced” tonsil, but at least this is a disease that has symptoms. Having a rare disease that presents with no symptoms that has a high mortality rate would truly be a bad scenario. I am glad to find out that this particular disease’s mortality rate for correctly-diagnosed cases has very much decreased to 5% and that the patient included in this case study was not included in the mortality rate. Thanks for sharing this relative information Kirstin!
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