Pathogenesis and Chemotherapy of Human Virus Infections
National Institute Of Allergy And Infectious Diseases
Investigators
Linked publications, trials & patents
Abstract
Epstein Barr virus (EBV) is associated with 200,000 cases of cancer each year worldwide and is the cause of infectious mononucleosis. The virus is associated with nasopharyngeal carcinoma, gastric carcinoma, Burkitt lymphoma, and Hodgkin disease. The virus causes lymphomas in some persons who have bone marrow or solid organ transplants. Immunodeficient patients with mutations in specific genes, can develop lymphoproliferation associated with EBV or EBV-positive B cell lymphomas. EBV is associated with some diseases in which the virus persists in T cells or NK cells. One of these diseases has been referred to as hydroa vacciniforme-like lymphoproliferative disorder. The classic form of this disease is indolent, self-limited, and more common in whites. The systemic form is severe and includes fever, lymphadenopathy, and often liver involvement, and it is more common in Asians and Latin Americans. In 2023, after consultation with several pathologists, based on clinical studies by our group and others, this disease has been renamed hydroa vacciniforme lymphoproliferative disorder by an International Consensus Clinical Advisory Committee since it is known now that all lesions from these patients contain EBV. EBV has also been associated with chronic active Epstein-Barr virus infection in which the virus infects B, T, or NK cells. After extensive discussions with pathologists, based on our clinical studies and those of others and because most adults are chronically infected with EBV, this disease has now been renamed chronic active Epstein-Barr virus (CAEBV) disease. Previously, CAEBV disease included patients with EBV-infected T, NK, or B cells; however, we have found that many patients that had been diagnosed with B cell CAEBV have underlying primary immunodeficiencies; therefore, patients with EBV in B cells are no longer classified as having chronic active EBV disease.
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