Chronic active Epstein-Barr virus disease
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Background
- Life-threatening infection with Epstein-Barr virus involving NK and T cells
Pathophysiology
- EBV infection involving B, T, or NK cells
Epidemiology
- Most cases reported in people of Asian and North American Indigenous descent
- Most common in Japan
Clinical Manifestations
- Symptoms include fever, liver dysfunction, splenomegaly, lymphadenopathy, and thrombocytopenia
- May also have hepatomegaly, anemia, mosquito bite hypersensitivity, rashes, oral ulcers, hemophagocytic lymphohistiocytosis, coronary artery aneurysm, liver failure, lymphoma, and interstitial pneumonia
- Occasionally has uveitis, CNS disease, intestinal perforation, and myocarditis
Management
- Hematopoietic stem cell transplantation is the only curative treatment
- Symptoms may be temporarily improved with corticosteroids
References
- ^ Hiroshi Kimura, Yo Hoshino, Hirokazu Kanegane, Ikuya Tsuge, Takayuki Okamura, Keisei Kawa, Tsuneo Morishima. Clinical and virologic characteristics of chronic active Epstein-Barr virus infection. Blood. 2001;98(2):280-286. doi:10.1182/blood.v98.2.280.
- ^ 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;117(22):5835-5849. doi:10.1182/blood-2010-11-316745.
- ^ Motohiko Okano, Keisei Kawa, Hiroshi Kimura, Akihiro Yachie, Hiroshi Wakiguchi, Akihiko Maeda, Shosuke Imai, Shouichi Ohga, Hirokazu Kanegane, Shigeru Tsuchiya, Tomohiro Morio, Masaaki Mori, Shumpei Yokota, Shinsaku Imashuku. Proposed guidelines for diagnosing chronic active Epstein-Barr virus infection. American Journal of Hematology. 2005;80(1):64-69. doi:10.1002/ajh.20398.
