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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

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A Forgotten Disease

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  1. A Forgotten Disease A Case Study about Lemierre’s Disease by Brandy Harkins

  2. 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

  3. 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

  4. 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)

  5. 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

  6. 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)

  7. 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?

  8. 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%

  9. 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

  10. 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)

  11. 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

  12. 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

  13. 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!

  14. 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

  15. 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.

  16. 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.

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