Chronic active EBV infection

Chronic active EBV infection (CAEBV) 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]

Characteristics

The most common symptoms of CAEBV include:[1][2][3][4]

Complications include:[1][2][4]

  • Interstitial pneumonia
  • Lymphoma, including B-cell, T-cell and NK-cell lymphomas[5]
  • Haemophagocytic syndrome
  • Coronary artery aneurysms
  • Liver failure
  • Nasopharyngeal carcinoma
  • Gastric adenocarcinoma
  • CNS
  • Intestinal perforation
  • Myocarditis
  • Peripheral neuropathy

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:[4][6]

There is also evidence supporting a role for TGF-β in the disease.[6] Those that develop the haemophagocytic syndrome often exhibit an abnormally high amount of IL-1β and IFN-γ.[7]

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][2][4][6]

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.[7] Factors indicative of a poor prognosis include: thrombocytopenia, late onset of the disease (age ≥ 8 years) and T cell involvement.[8]

References

  1. 1 2 3 4 5 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 3112034Freely accessible. PMID 21454450.
  2. 1 2 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.
  3. ↑ 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.
  4. 1 2 3 4 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. PMID 19236801.
  5. ↑ 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.
  6. 1 2 3 Cohen, JI (June 2009). "Optimal treatment for chronic active Epstein-Barr virus disease." (PDF). Pediatric transplantation. 13 (4): 393–396. doi:10.1111/j.1399-3046.2008.01095.x. PMC 2776035Freely accessible. PMID 19032417.
  7. 1 2 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.
  8. ↑ 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. PMID 12599068.
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