Epstein–Barr virus–associated lymphoproliferative diseases

Epstein–Barr Virus–Associated Lymphoproliferative Diseases (EBV-LPDs) are a group of disorders caused by the Epstein–Barr virus (EBV), a member of the Herpesviridae family. These diseases range from benign to malignant lymphoproliferative disorders and affect both immunocompetent and immunocompromised individuals. EBV is best known for causing infectious mononucleosis, but its association with various forms of cancer and autoimmune diseases has been increasingly recognized.

Etiology and Pathogenesis

EBV infects B cells and epithelial cells, establishing a lifelong latent infection. The virus is transmitted through saliva and can lead to a variety of clinical manifestations. In immunocompetent hosts, primary EBV infection is usually asymptomatic or causes infectious mononucleosis. However, in immunocompromised individuals, such as those with HIV/AIDS, post-transplant patients on immunosuppressive therapy, and individuals with genetic immunodeficiencies, EBV can lead to uncontrolled B-cell proliferation, resulting in lymphoproliferative diseases.

Classification

EBV-associated lymphoproliferative diseases can be classified into several types, including but not limited to:

• Burkitt lymphoma
• Hodgkin lymphoma
• Post-transplant lymphoproliferative disorder (PTLD)
• Primary central nervous system lymphoma (PCNSL)
• Chronic active EBV infection (CAEBV)
• Hemophagocytic lymphohistiocytosis (HLH) associated with EBV

Clinical Manifestations

The clinical presentation of EBV-LPDs varies widely, from asymptomatic to life-threatening conditions. Symptoms depend on the type of disease, the organs involved, and the patient's immune status. Common symptoms include fever, lymphadenopathy, weight loss, and night sweats.

Diagnosis

Diagnosis of EBV-associated lymphoproliferative diseases involves a combination of clinical assessment, laboratory tests, imaging studies, and histopathological examination. Serological tests for EBV antibodies and PCR for EBV DNA can help confirm the diagnosis. Biopsy of affected tissues is often necessary to differentiate between the various types of lymphoproliferative disorders.

Treatment

Treatment strategies for EBV-LPDs depend on the specific disease, its stage, and the patient's overall condition. Options may include antiviral therapy, immunotherapy, chemotherapy, radiation therapy, and, in some cases, stem cell transplantation. Management of immunocompromised patients also involves adjusting immunosuppressive medications to reduce the risk of EBV-driven proliferation.

Prognosis

The prognosis of EBV-associated lymphoproliferative diseases varies significantly among different disorders and individual patients. Early diagnosis and appropriate treatment can improve outcomes, especially in diseases like Burkitt lymphoma and Hodgkin lymphoma. However, some conditions, such as PTLD and CAEBV, have a more challenging prognosis.

Prevention

Preventive measures against EBV infection are limited. Good hygiene practices, such as avoiding sharing drinks and utensils, can reduce the risk of EBV transmission. For individuals at high risk of developing EBV-LPDs, such as transplant recipients, monitoring for EBV DNA levels and adjusting immunosuppressive therapy may help prevent disease development.