Chronic active EBV infection

Chronic active EBV infection or in its expanded form, chronic active Epstein–Barr virus infection is a very rare and often fatal complication of Epstein–Barr virus (EBV) infection that most often occurs in children or adolescents of Asian or South American lineage, although cases in Hispanics, Europeans and Africans have been reported.[1] It is classified as one of the Epstein-Barr virus-associated lymphoproliferative diseases (i.e. EBV+ LPD).[2]

Chronic active EBV infection
Other namesCAEBV

Presentation

The most common symptoms of CAEBV include:[1][3][4][5]

Complications include:[1][3][5]

Pathophysiology

It arises from the cells that constitute the immune system, most often the T-cells and NK cells in Asians/South Americans and the B-cells in the other racial groups.[1] Various cytokine anomalies have been reported in people with CAEBV, examples include:[5][7]

There is also evidence supporting a role for TGF-β in the disease.[7] Those that develop the haemophagocytic syndrome often exhibit an abnormally high amount of IL-1β and IFN-γ.[8]

Diagnosis

Treatment

The only known cure for CAEBV is allogenic haematopoietic stem cell transplant (HSCT), with all other treatment options (rituximab, cytotoxic chemotherapy and immunosuppressive therapy) being nothing more than stopgaps.[1][3][5][7]

Prognosis

Without HSCT the condition is inevitably fatal and even HSCT is no guarantee, with a significant portion of patients dying from the disease progression.[8] Factors indicative of a poor prognosis include: thrombocytopenia, late onset of the disease (age ≥ 8 years) and T cell involvement.[9]

References
  1. Cohen, JI; Jaffe, ES; Dale, JK; Pittaluga, S; Heslop, HE; Rooney, CM; Gottschalk, S; Bollard, CM; Rao, VK; Marques, A; Burbelo, PD; Turk, SP; Fulton, R; Wayne, AS; Little, RF; Cairo, MS; El-Mallawany, NK; Fowler, D; Sportes, C; Bishop, MR; Wilson, W; Straus, SE (31 March 2011). "Characterization and treatment of chronic active Epstein-Barr virus disease: a 28-year experience in the United States" (PDF). Blood. 117 (22): 5835–5849. doi:10.1182/blood-2010-11-316745. PMC 3112034. PMID 21454450.
  2. Rezk SA, Zhao X, Weiss LM (June 2018). "Epstein - Barr virus - associated lymphoid proliferations, a 2018 update". Human Pathology. 79: 18–41. doi:10.1016/j.humpath.2018.05.020. PMID 29885408.
  3. Kimura, H; Hoshino, Y; Kanegane, H; Tsuge, I; Okamura, T; Kawa, K; Morishima, T (15 July 2001). "Clinical and virologic characteristics of chronic active Epstein-Barr virus infection" (PDF). Blood. 98 (2): 280–286. doi:10.1182/blood.V98.2.280. PMID 11435294.
  4. Gotoh, K; Ito, Y; Shibata-Watanabe, Y; Kawada, J; Takahashi, Y; Yagasaki, H; Kojima, S; Nishiyama, Y; Kimura, H (15 May 2008). "Clinical and virological characteristics of 15 patients with chronic active Epstein-Barr virus infection treated with hematopoietic stem cell transplantation" (PDF). Clinical Infectious Diseases. 46 (10): 1525–34. doi:10.1086/587671. PMID 18419486.
  5. Lu, G; Xie, ZD; Zhao, SY; Ye, LJ; Wu, RH; Liu, CY; Yang, S; Jin, YK; Shen, KL (5 February 2009). "Clinical analysis and follow-up study of chronic active Epstein-Barr virus infection in 53 pediatric cases" (PDF). Chinese Medical Journal. 122 (3): 262–6. doi:10.3760/cma.j.issn.0366-6999.2009.03.005 (inactive 2019-10-16). PMID 19236801.
  6. Ohtsuka, R; Abe, Y; Sada, E; Kiyasu, J; Ashikari, A; Shiratsuchi, M; Nishimura, J; Takayanagi, R; Ohshima, K (2009). "Adult patient with Epstein-Barr virus (EBV)-associated lymphoproliferative disorder: chronic active EBV infection or de novo extranodal natural killer (NK)/T-cell lymphoma, nasal type?" (PDF). Internal Medicine. 48 (6): 471–4. doi:10.2169/internalmedicine.48.1346. PMID 19293549.
  7. Cohen, JI (June 2009). "Optimal treatment for chronic active Epstein-Barr virus disease". Pediatric Transplantation. 13 (4): 393–396. doi:10.1111/j.1399-3046.2008.01095.x. PMC 2776035. PMID 19032417.
  8. Kimura, H; Hoshino, Y; Hara, S; Sugaya, N; Kawada, J; Shibata, Y; Kojima, S; Nagasaka, T; Kuzushima, K; Morishima, T (15 February 2005). "Differences between T cell-type and natural killer cell-type chronic active Epstein-Barr virus infection" (PDF). The Journal of Infectious Diseases. 191 (4): 531–539. doi:10.1086/427239. PMID 15655776.
  9. 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; Japanese Association for Research on Epstein-Barr Virus and Related Diseases (15 February 2003). "Prognostic factors for chronic active Epstein-Barr virus infection" (PDF). The Journal of Infectious Diseases. 187 (4): 527–533. doi:10.1086/367988. hdl:10126/4219. PMID 12599068.
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