Lemierre’s Disease

Lemierre’s Disease
Presentation by:by Brandy Harkins

Patient Presentation
* 20 year old female
* Diagnosed with infectious mononucleosis 2 days prior to admission
* No remarkable previous medical history
* Blood pressure – 101/72
* Pulse – 167 beats/min
* Respiratory rate – 52/min
* Shortness of breath and chest pain with shallow breathing
* Sore throat
* Headache
* Fever
* Decreased appetite
* Abdominal pain (no nausea, vomiting, diarrhea or constipation)
* Pale
* Initial diagnosis was pneumonia

Laboratory Findings
* Blood culture positive at 24hrs (Fusobacterium necrophorum)
* Monospot negative
* EBV-VCA IgG positive
* Increased fibrinogen, PT & PTT
* Increased bilirubin
* Liver enzymes – AST 74 (19-45), ALT 44 (8-37)
* WBC’s – 15.3 (4.0-10.9)
* Plts – 106 (150-400)

Fusobacterium necrophorum
* Normal flora in oral cavity, female genital tract, and gastrointestinal tract
* Pleomorphic gram negative bacillus (GNB)
* Non-motile
* Non-spore forming
* Strict anaerobe

Disease Association
* Can cause parotitis, otitis media, sinusitis, odontogenic infection, mastoiditis and Lemierre’s syndrome (necrobacillosis)
* Produces lipopolysaccharide endotoxin, hemagglutinin, leukocidin, and hemolysin
* Invasion usually from intra-oral disease (bacterial tonsillitis, EBV, dental disease)

Questions to Consider
* What organism is usually responsible for Lemierre’s sydrome?
* Why has Lemierre’s become the “forgotten disease?”
* What are the symptoms of the syndrome?
* What age group is most commonly affected?
* What are the stages commonly seen with Lemierre’s and at which stage does the red flag appear?

Lemierre’s Syndrome
* Thrombophlebitis of the internal jugular vein (IJV) due to anaerobic infection (usually F. necrophorum)
* Virulent toxin production with platelet aggregation IJV thrombosis
* Causes severe disease as primary pathogen in healthy individuals
* Generally affects young adults 16-29 y/o
* 1 in 1,000,000 infected per year
* Common in the early 20th century, but disappeared with antibiotics
* Used to have 100% mortality rate…today’s rate is 6-20%

Disease Presentation
* Sore throat
* Tender/swollen lymph nodes
* Prolonged fever
* May experience abdominal pain, nausea or vomitting
* Bacteremia
* Increased WBC’s or left shift
* Hyperbilirubinemia and slight increase in liver enzymes

Classical Characterization
* Primary infection in oropharynx
* Septicemia documented by at least one positive blood culture bottle
* Evidence of internal jugular vein thrombosis
* At least one metastatic focus (usually pulmonary)

Stages
* Patient generally exhibits three stages
1. Pharyngitis – sore throat (< 1 week)
2. Local invasion of lateral pharyngeal space and IJV septic thrombophlebitis swollen/tender neck = red flag
3. Metastatic complications – fever, pulmonary infiltrates or possible joint involvement

Treatment
* Fatal if untreated
* 1-2 weeks IV antibiotics and 2-4 weeks oral antibiotics
* Aggressive approach when patient has pharyngitis and tender/swollen neck
o Get blood culture
o Look for evidence of IJV thrombophlebitis with CT, MRI, ultrasound
o Use antibiotics affective against anaerobes (clindamycin, metronidazole, etc.)
* Anticoagulant therapy controversial
* May require surgery to remove the IJV because of continuing sepsis, localized collection of pus, or embolism

So why’s it so hard to diagnose?
* Rarely seen in the antibiotic-era…most physicians have never seen it
* Can present with pneumonia-like or meningitis-like clinical picture
* Many sore throats have a viral etiology and are not treated with antiobiotics, therefore a patient can be misdiagnosed and untreated for long periods of time before clinicians suspect Lemierre’s
* More severe with longer duration of symptoms than viral sore throat!

Summary
* Lemierre’s syndrome is usually caused by Fusobacterium necrophorum
* Affects healthy young adults
* Patient presents with fever, sore throat, swollen/tender neck (red flag)
* 3 stages – pharyngitis, IJV thrombosis, and metastatic complications
* Disease severity is often underestimated and left untreated or is treated as a case of pneumonia or meningitis


References
Credits:This case study was created by Brandy Harkins, MT(ASCP) while she was a Medical Technology student in the 2004 Medical Technology Class at William Beaumont Hospital, Royal Oak, MI.

Lemierre’s Disease.ppt