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EBV-Related Lymphomas

Last update: 11/10/2012

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The evidence connecting Epstein Barr virus (EBV) with some kinds of lymphoma is building.  Tere is a strong association between EBV an Burkitt's lymphoma, but other factors may also be needed for a cancer to develop, such as suppressed immunity.  

However, being EBV-positive (a very common virus) does not mean you will develop a lymphoma, or that you are at considerably high risk.

About Epstein Barr Virus: Evidence is building that Epstein-Barr virus can "change normal cells into cancerous ones." "That virus already is known to cause infectious mononucleosis in humans and has been associated with such malignancies as Burkett’s lymphoma, Hodgkin’s lymphoma and nose and throat cancer. It is especially hazardous to AIDS patients and other patients whose immune systems have weakened."  unc.edu 

"The Epstein Barr virus (EBV) was the first described oncovirus (cancer causing virus), which has been associated with the development of a variety of lymphoproliferative disorders, such as Burkitt, primary CNS, NK/T-cell, plasmablastic and Hodgkin lymphoma. 

EBV infection occurs early in childhood, and approximately 90 to 95% of adults worldwide are EBV-seropositive. 

EBV expression has also been reported in patients with diffuse large B-cell lymphoma (DLBCL). " (Beltran, et al.) ncbi.nlm.nih.gov/

Medinfo.edu: Technical background on EBV

"Epstein-Barr virus was discovered by observation of herpes-like virions in electron micrographs of biopsies of Burkitt's lymphoma. It was later identified as the cause of infectious mononucleosis and nasopharyngeal carcinoma. 

In immunodeficient individuals it can cause B-cell lymphomas. The receptor for EB virus is also the receptor for the C3d component of the complement system. It is expressed only on B-cells and epithelial cells of the oropharynx and nasopharynx. Infection of the epithelial cells of the oro- and nasopharynx is permissive. 

Virus is shed into the saliva and infects B lymphocytes in lymphatic tissue and blood. Infection of B cells can cause a latent infection, stimulate their growth, or immortilize the cells. In mononucleosis the characteristic lymphocytosis is due to activation and proliferation of supressor (CD8) T cells. This causes the swollen lymph glands as well as the hepato- and splenomegaly. 

The disease is quite mild in children but more severe in adolescents and adults. It produces life-long immunity. Thus a good strategy for control of disease would be to ensure infection of children at a young age. Clinical diagnosis of mononucleosis is usually based on the presence of lymphocytosis, atypical lymphocytes, pharyngitis, and the presence of heterophile antibody (Ab to sheep and horse RBC's), which is the basis for the monospot test."  medinfo.ufl.edu

"In normal individuals, latent EBV infection is controlled by humoral immunity, cytotoxic T cells, and the interferon (IFN) system (Jabs et al., 1996)."  uq.edu.au 

Types of lymphomas associated with EBV

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AIDS-related lymphomas  PAL | meb.uni-bonn.de/cancer.gov
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Burkitt's Lymphoma  PAL
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Cutaneous Lymphoma Associated With Epstein-Barr Virus Infection in 2 Patients Treated With Methotrexate  mayo.edu
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EBV-associated primary central nervous system lymphoma  ASCO
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Epstein-Barr virus polymorphic B-cell lymphoma associated with leukemia and with congenital immunodeficiencies  jco.org
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Hodgkin's Lymphoma  PAL
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Characteristics of Hodgkin's Lymphoma after Infectious Mononucleosis

NEJM: Characteristics of Hodgkin's Lymphoma after Infectious Mononucleosis

http://www.nejm.org/doi/full/10.1056/NEJMoa023141#t=article

snips:

"We estimated that the median incubation period for Hodgkin's lymphoma attributable to infectious mononucleosis–related EBV infection was 4.1 years, with a peak in risk 2.4 years after infection.

These estimates are in accordance with those of a large case–control study of Hodgkin's lymphoma in which the median interval between infectious mononucleosis and Hodgkin's lymphoma was five years.17

...

We emphasize that in absolute terms, the risk of Hodgkin's lymphoma after infectious mononucleosis is only on the order of 1 case per 1000 persons.5  Consequently, other cofactors acting in concert with infectious mononucleosis–related EBV infection presumably must be present for the infection to give rise to Hodgkin's lymphoma.16

 

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Diffuse Large B-Cell Lymphomas

The impact of Epstein-Barr virus status on clinical outcome in diffuse large B-cell lymphoma. (Park, et al)  http://bloodjournal.hematologylibrary.org/cgi/content/full/110/3/972 
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Other - many types of lymphoma can be EBV-positive
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Post-transplantation lymphoma  tpis.upmc.edu
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T-/NK-cell lymphoma  PAL

Staging: Staging refers to the how widespread the disease is. Imaging tests (CT MRI, PET, Gallium) and bone marrow biopsies are commonly done to estimate this.  See Staging for more detail.

Treatment: The type of treatment will depend on the type of lymphoma, how aggressive it is, and other clinical details, such as the patient's age, general health, and the number and types of prior treatments. Also see Treatment Decisions

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Research News

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Immunotherapy Demonstrates Long-Term Success in Treating EBV+ Lymphoma http://bit.ly/71qLT
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A phase 1/2 trial of arginine butyrate and ganciclovir (anti-viral) in patients with EBV–associated lymphomas http://bit.ly/3g1dn6  
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Combination antiviral therapy for EBV-associated primary central nervous system lymphoma  ASCO
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Epstein-Barr virus associated diffuse large B-cell lymphoma complicated by autoimmune hemolytic anemia and pure red cell aplasia. 
Leuk Lymphoma. 2001 Jul;42(3):539-42. PMID: 11699422
 
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