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Hodgkin lymphoma

 

 

Etiology

 

The etiology of Hodgkin lymphoma is unknown. 

Disease is most likely linked with:

  • Prior viral infection
  • Genetic predisposition

 

Viral Environmental Factor - EBV

Prior exposure to Epstein-Barr Virus (EBV) is linked to development of Hodgkin lymphoma.

Exposure to EBV as an important etiologic component is supported by:

  • Detection of EBV viral genome incorporated into human DNA in Reed-Sternberg cells.
  • A gene found in Reed-Sternberg cell that codes for a latent membrane protein inducing cell transformation was identified as viral DNA from EBV.  Possible development of the disease when this latent gene becomes activated.
  • More than 50% of childhood cases are EBV-positive.
  • 100% of HIV associated Hodgkin lymphoma are EBV-positive.  The number of EBV-positive cases varies among different histological subtypes, with mixed-cellularity type being most strongly correlated.  

Studies show a clustering effect in incidences of Hodgkin lymphoma based on geographic patterns in young adults but not older groups:

  • Exposure to EBV at a specific age is an etiologic factor. 
  • Infection during adolescence is associated with subsequent development of mononucleosis.
  • Infection during childhood is associated with Burkitt lymphoma. 
  • Patients with history of mononucleosis have a higher chance of developing Hodgkin lymphoma within five years.

However - EBV is a common infection and is associated with other malignancies; Hodgkin disease is not a common malignancy.  Many people infected with EBV will not develop lymphoma. 

Hodgkin lymphoma is probably the result of altered immunity in a chronically immunodeficient individual stimulated by viral factors.

Genetic Predisposition

Less than 1% of Hodgkin lymphoma is associated with a family history of this disease. 

  • Siblings of patients have a 3-7-fold increase in future development of Hodgkin lymphoma, especially same gender siblings.  
  • A higher risk (99-fold increase) in monozygotic twins but not dizygotic twins.

Individuals with inherited or pre-existing immunodeficiency disease are more susceptible to developing Hodgkin lymphoma.  Increased incidence rates are seen in:

Hodgkin lymphoma has a weaker genetic predisposition compared to other lymphomas.

 

 

 

 

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