MONONUCLEOSIS

 

MONONUCLEOSIS

 

DEFINITION:

A syndrome caused by the Epstein Barr Virus (EBV) resulting in a sore throat, fever, and fatigue.

EPIDEMIOLOGY:

  • incidence: ?
  • age of onset:
    • peak incidence in adolescence (60-80% are seropositive)
    • rare in children < 2 years of age
  • risk factors:
    • immunocompromised hosts

PATHOGENESIS:

1. Background

1. Ebstein Barr Virus

  • a member of the herpes group of viruses
  • routes of infection:
    • primarily via oral secretions
    • also parenterally (transfusions, organ transplants)
    • sexual transmission
  • incubation period: 30-50 days
  • enters the body via oral mucosa cells -> targets B-lymphocytes -> transformed into freely dividing B-cells (the heterophile antibodies are directed towards these immortilized cells)
  • host responds to the EBV invasion by 2 populations of T-Cells: Natural Killer Cells (the 'atypical lymphocytes') and Lymphokines
  • the clinical presentation of an EBV infection is primarily a function of the age-dependent immunological response to the virus
  • poorly contagious with a low secondary attack rate
  • immunosuppressed patients who are seropositive often reactivate the EBV and 60% will shed the virus - other infectious agents producing a mono-like illness:
    • CMV, HIV, toxoplasmosis, hepatitis A, adenovirus, rubella, mumps, diphtheria, group A beta-hemolytic streptococcus

CLINICAL FEATURES:

1. Infectious Manifestations

  • insidious onset with symptoms lasting 2-4 weeks followed by a gradual recovery

1. Symptoms

  • sore throat
  • fever (39 C)
  • fatigue, malaise, poor appetite
  • headache

2. Signs

  • pharyngitis
    • exudative with palatal petechiae
    • marked tonsillar enlargement +/- exudates
  • lymphadenopathy
    • non-tender
    • unilateral or bilateral
    • anterior and posterior cervical
    • also epitrochlear and generalized
  • splenomegaly (50%)
  • +/- LUQ tenderness
  • rubella-like rash (3-15%)
    • develops in 80-90% of patients given ampicillin or amoxicillin
  • others
    • hepatomegaly (33%)
    • jaundice (5%)
    • periorbital edema

2. Complications

1. Neurological

  • Aseptic Meningitis, Encephalitis, Cerebellitis
  • Myelitis, Transverse Myelitis
  • Guillain-Barre Syndrome, Peripheral Neuropathies, Bell Palsy
  • acute ataxia, chorea, seizures, nuchal rigidity

2. Respiratory

  • acute upper airway obstruction (tonsillomegaly or subglottic edema)
  • interstitial pneumonitis

3. Cardiovascular

  • myocarditis

4. Gastrointestinal

  • splenic rupture
  • anicteric hepatitis (80%)
  • pancreatitis
  • parotitis
  • Reye Syndrome

5. Genitourinary

  • orchitis

6. Hematological

  • autoimmune hemolytic anemia - I & II
  • thrombocytopenic purpura
  • pancytopenia, aplastic anemia
  • Hemophagocytic Syndrome

7. Neoplastic

  • Lymphoproliferative Syndrome (Duncan Disease)
  • Burkitt Lymphoma
  • Nasopharyngeal Carinoma
  • ? Hodgkin's Lymphoma

INVESTIGATIONS:

1. CBC + Smear

  • hemolytic anemia (positive Coombs test)
  • leukocytosis (with 'atypical lymphocytosis [T-cells])
  • neutropenia, thrombocytopenia

2. Serum

  • mildly elevated AST, ALT, & bilirubin (anicteric hepatitis)

3. Antibodies

1. Heterophil

  • a + test is pathognomonic
  • Paul-Bunnell-Davidsohn test
  • Monospot (Agglutination Test)
  • the heterophil-antibody response is a function of age and may be absent in infants & young children (thus a negative monospot in a child <10 years of age should be followed up with a specific EBV antibody test

2. Specific EBV

1. Acute Infection

  • positive Virus Capsid Antigen (VCA)-IgM
  • positive anti-VCA-IgG
  • negative Ebstein Barr Nuclear Antigen (EBNA)

MANAGEMENT:

1. Supportive

  • signs and symptoms resolve over 2-4 weeks followed by a gradual recovery
  • avoid activities that could lead to splenic rupture, i.e., contact sports, bike riding, skating, skiing, etc. until the splenomegaly is no longer detected

2. Medical (for complications)

1. Steroids

1. Prednisone

  • indicated for upper airway obstruction due to tonsillar enlargement

2. Systemic

  • indicated for neurolgical and hematological complications

2. Antibiotics

1. Group A beta-hemolytic Streptococcus

  • use erythromycin or clindamycin for a secondary infection and avoid ampicillin and amoxil

 

 

Pediatric Database - MONONUCLEOSIS

Pediatric Organization - Pedbase [at] Gmail.com