A Look at Lemierre’s A Forgotten Disease

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Transcript A Look at Lemierre’s A Forgotten Disease

A Forgotten Disease
A Case Study about Lemierre’s Disease
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
Presentation – continued …
 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
1.
2.
3.
4.
5.
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
– Get blood culture
– Look for evidence of IJV thrombophlebitis with CT, MRI,
ultrasound
– 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
1. Chirinos J et al. The evolution of Lemierre’s syndrome: report of 2
cases and review of the literature. Medicine. 2002;81:458.
2. Deadly sore throat ailment on the rise in UK. Clinical Infectious
Diseases. 2002;35:1.
3. Harrison’s Online. www.harrisons.accessmedicine.com
4. Moore B, Dekle C, Werkhaven J. Bilateral Lemierre’s syndrome: a
case report and literature review. Ear, Nose and Throat Journal.
2002;81:234.
5. Singhal A, Morris D. Lemierre’s syndrome. Southern Medical
Journal. 2001;94:886.
6. Woywodt A et al. A swollen neck. The Lancet. 2002;360:1838.
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.