Epstein Barr Syndrome

What is Epstein Barr Syndrome?

Epstein Barr Syndrome is a viral infection caused by Epstein Barr Virus (EBV). Epstein-Barr Virus is a type of membrane herpesvirus. Epstein-Barr Virus are widespread globally and children are most susceptible to Epstein-Barr infection but can occur at any time of life. The acute infection does not cause much harm, as immunity develops against this virus. The scientific paper reveals that almost 95% worldwide population The most common associated infection of Epstein Barr Virus in an adult is mononucleosis¹.

Chronic Epstein Barr Syndrome

In rare cases, Epstein Barr Syndrome causes active EBV disease (CAEBV) and outcome of this disease causes a life-threatening condition. Japanese are more vulnerable for CAEBV. The cause of CAEBV is related to complications arises during acute EBV infection and that persist for prolong period. CAEBV is progressive in nature, therefore symptoms are aggravated with increasing time span. The disease condition is progressive due to EBV DNA levels and viral RNA and proteins become high in the blood and in tissues respectively.

Depending on the finding of EBV in blood cells, CAEBV is further classified. The research data showed the possible interaction of EBV with different cells are B cells, T cells, and Natural killer cells. This interaction can lead to altered gene mutation. Involvement of B cells causes lytic infection².

Causes

Epstein Barr Syndrome is a viral infection and the causative organism of this syndrome is Epstein Barr Virus also known as herpes 4 or human herpes.

Symptoms

The symptoms of Epstein Barr Syndrome are depending on when the infection develops and type of infection.

Acute

Acute Epstein Barr Syndrome arises at infant and childhood usually, does not show any specific symptoms or asymptomatic. All the symptoms related to acute Epstein Barr Syndrome are mild and self-limiting, but the virus can stay life-long and can reactivate in any stage of life. The different included symptoms are as follows³:

• Fatigue
• Headache
• Fever
• A sore throat due to inflammation of throat
• Lymphadenopathy means swelling of lymph nodes in the neck
• Splenomegaly means enlargement of spleen
• Hepatomegaly or liver inflammation
• Rash

Chronic

In the case of chronic Epstein Barr Syndrome, apart from above mentioned symptoms, some other included features are hepatic dysfunction, anemia, thrombocytopenia, hypersensitivity to mosquito bites, oral ulcers, hemophagocytic syndrome, lymphoma, coronary artery aneurysms, interstitial pneumonia, liver failure⁴.

Less commonly following features are also included

• CNS disease
• Myocarditis
• Intestinal perforation
• Uveitis
• Calcification of basal ganglia

How do you get Epstein Barr Syndrome?

The causative virus of Epstein-Barr Syndrome can easily spread and that can cause the easy onset of infection, especially in infants and children.

• The most common media through which EBV virus can transmit is a bodily fluid, such as saliva. Kissing and sharing food is a most common way to transmit the infection.
• Other modes of transmission of infections are included blood transfusions, semen, and blood during intercourse and organ transplantations.
• Even non-living objects or contaminated useable of infected patients (fomits) like utensils, toothbrush etc can also assist in transmitting the infection. The moist place or objects can assist to survive the virus for prolong period.
• EBV reaches body compartment and a certain period of time unable to provide any symptoms. during this period EBV can act as a carrier and infect others. The virus is latent or inactive in state after reaching n the body system and any favorable condition reactivate then and able to transmit the infection even after a protract time interval³.
• EBV infection usually cannot be transmitted to the fetus during pregnancy, even if the mother is infected. There is no clinical history of birth defect in associated with EBV infected pregnant women delivery⁵.

Diagnosis

Epstein-Barr Syndrome can be detected by laboratory tests, which can identify the presence of antibody in the blood and provided report can reveal the recent or past history of EBV infection. The following EBV based antigen tests are performed in the laboratory to detect the corresponding antibody.

• Viral Capsid Antigen
• Early antigen
• Epstein-Barr Syndrome Diagnosis
• Monospot test

Depending upon this test results, the clinician can interpret susceptibility to infection, recent onset of infection and past history of infection³.

Treatment

There is no specific treatment recommendation for Epstein Barr Syndrome.

• Doctors prescribe corticosteroids to treat severe throat swelling and also spleen enlargement. But most of the acute Epstein Barr Syndrome symptoms are self-limiting, therefore the requirement of steroid therapy is rare. Different antiviral drugs like acyclovir, ganciclovir or foscarnet are available in the market to treat oral hairy leukoplakia.
• Prolonged follow-up of treatment is also usually not require, but in cases of enlargement of spleen follow up is require until the condition recover completely. In neurological complications, consultation with a neurologist is required.
• Some general therapeutic recommendations, such as adequate rest, sufficient fluid intake and analgesic to control pains are beneficial to minimizing the Epstein-Barr Syndrome symptoms and associated complication.
• Contact sports activity is restricted for affected patient, as it may cause harm for enlargement of the spleen.

Prognosis

Epstein-Barr Syndrome is mostly self-limiting and provides mild symptoms. Therefore, acute Epstein Barr Syndrome has a good prognosis. But some complications can arise with Epstein-Barr Syndrome, which includes a number of malignancies, such as B cell lymphomas, Hodgkin’s disease, and nasopharyngeal carcinoma. Prognosis of malignancy depends upon certain factors like a stage, time of detection, commencement of treatment.

Prognosis is also poor in presence of thrombocytopenia, the onset of disease at a later stage of life or after 8 years of age, EBV associated with T cells infection. Mortality is common due to opportunistic infections, liver failure or malignant lymphoma⁶.

References

1. Jeffrey I. Cohen; Optimal Treatment for Chronic Active Epstein-Barr Virus Disease; Pediatr Transplant. Author manuscript; available in PMC 2010 Jun 1; Published in final edited form as: Pediatr Transplant. 2009 Jun; 13(4): 393–396. doi: 10.1111/j.1399-3046.2008.01095.x; https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2776035/
2. Jeffrey I. Cohen, Elaine S. Jaffe, Janet K. Dale, Stefania Pittaluga, Helen E. Heslop, Cliona M. Rooney, Stephen Gottschalk, Catherine M. Bollard, V. Koneti Rao, Adriana Marques, Peter D. Burbelo, Siu-Ping Turk, Rachael Fulton, Alan S. Wayne, Richard F. Little, Mitchell S. Cairo, Nader K. El-Mallawany, Daniel Fowler, Claude Sportes, Michael R. Bishop, Wyndham Wilson, Stephen E. Straus; Characterization and treatment of chronic active Epstein-Barr virus disease: a 28-year experience in the United States; Blood. 2011 Jun 2; 117(22): 5835–5849. Prepublished online 2011 Mar 31. doi: 10.1182/blood-2010-11-316745; https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3112034/
3. Epstein-Barr Virus and Infectious Mononucleosis; Centers For Disease Control & Prevention; https://www.cdc.gov/epstein-barr/about-ebv.html
4. KIMURA H, HOSHINO Y, KANEGANE H, TSUGE I, OKAMURA T, KAWA K, MORISHIMA T. Clinical and virologic characteristics of chronic active Epstein-Barr virus infection. Blood. 2001;98:280–6.
5. John P. Cunha, (2016); Epstein-Barr Virus Infection; http://www.emedicinehealth.com/epstein-barr_virus_infection/page5_em.htm
6. KIMURA H, MORISHIMA T, KANEGANE H, OHGA S, HOSHINO Y, MAEDA A, IMAI S, OKANO M, MORIO T, YOKOTA S, TSUCHIYA S, YACHIE A, IMASHUKU S, KAWA K, WAKIGUCHI H. Prognostic factors for chronic active Epstein-Barr virus infection. J Infect Dis. 2003;187:527–33.