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 ( )  Plts – 106 ( )

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. What organism is usually responsible for Lemierre’s sydrome? 2. Why has Lemierre’s become the “forgotten disease?” 3. What are the symptoms of the syndrome? 4. What age group is most commonly affected? 5. 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 y/o  1 in 1,000,000 infected per year  Common in the early 20 th 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: Deadly sore throat ailment on the rise in UK. Clinical Infectious Diseases. 2002;35:1. 3. Harrison’s Online Moore B, Dekle C, Werkhaven J. Bilateral Lemierre’s syndrome: a case report and literature review. Ear, Nose and Throat Journal. 2002;81: Singhal A, Morris D. Lemierre’s syndrome. Southern Medical Journal. 2001;94: 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.