Clinical Analysis of Chronic Active Epstein-Barr Virus Infection_West China Medical Journal

Authors：

 LUJia-Jie , TANGGuang-Min ,  TANGHong

Center of Infectious Diseases, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;

Corresponding?author：

TANGHong, Email: htang6198@hotmail.com

Keywords：

Epstein-Barr virus infections; Lymphoproliferative disorders; Real time polymerase chain reaction

DOI：

10.7507/1002-0179.20140086

Video：

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Objective To improve the understanding of the diagnosis and therapy of chronic active Epstein-Barr virus (CAEBV) infection. Methods Data of 9 cases of CAEBV infection diagnosed between October 2008 and January 2013 were analyzed retrospectively,including clinical and auxiliary examination results,pathological data,especially EB virus (EBV) antibodies and DNA in peripheral blood mononuclear cells (PBMC) and infected tissue,and follow-up information. Results The major manifestations of the 9 patients were fever,splenomegaly,hepatomegaly,lymphadenopathy,and others,including general fatigue,nausea,skin rash,jaundice,and so on.The abnormalities of auxiliary examination were as follows:anemia,leucopenia,neutropenia,thrombocytopenia,elevated LDH and HBDH levels,liver dysfunction and abnormal chest CT findings.EBV serologic tests revealed high IgA antibody levels against EB viral capsid antigen (VCA) in 6 patients,and 8 patients had positive IgG antibody levels against early D antigen (EAD).The mean load of EBV-DNA detected by real time polymerase chain reaction (PCR) in the PBMC was 3.07×105 copies/mL.Six of the nine patients presented a poor clinical course.One of them died of intracranial hemorrhage,one of them died of multiple organ failure,one of them died of EBV-associated hemophagocytic syndrome,and one of them died of severe pulmonary infection.Four patients developed lymphoma.One of them died of hepatic failure and one of them died of severe infection in the process of anti-tumor treatment. Conclusion The clinical feature of CAEBV infection is varied.More attention should be paid to the disease because of its severe complications,poor prognosis and high mortality.

Citation： LUJia-Jie, TANGGuang-Min, TANGHong. Clinical Analysis of Chronic Active Epstein-Barr Virus Infection. West China Medical Journal, 2014, 29(2): 282-285. doi: 10.7507/1002-0179.20140086 Copy

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2.	Kimura H,Hoshino Y,Kanegane H,et al.Clinical and virologic characteristics of chronic active Epstein-Barr virus infection[J].Blood,2001,98(2):280-286.
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6.	Yamashita N,Kimura H,Morishima T.Virological aspects of Epstein-Barr virus infections[J].Acta Med Okayama,2005,59(6):239-246.
7.	宋紅梅,吳曉燕,王薇,等.嚴重慢性活動性EB病毒感染12例臨床回顧與隨訪[J].中華兒科雜志,2009,47(9):682-686.
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13.	Sakai Y,Ohga S,Tonegawa Y,et al.Interferon-alpha therapy for chronic active Epstein-Barr virus infection:potential effect on the development of T-lymphoproliferative disease[J].J Pediatr Hematol Oncol,1998,20(4):342-346.
14.	Fujisaki T,Nagafuchi S,Okamura T.Gamma-interferon for severe chronic active Epatein-Barr virus[J].Ann Intern Med,1993,118(6):474-475.
15.	Kawa-Ha K,Franco E,Doi S,et al.Successful treatment of chronic active Epstein-Barr virus infection with recombinant interleukin-2[J].Lancet,1987,1(8525):154.
16.	Kimura H.Pathogenesis of chronic active Epstein-Barr virus infection:Is this an infectious disease,lymphoproliferative disorder,or immunodeficiency [J].Rev Med Virol,2006,16(4):251-261.
17.	Imashuku S,Hibi S,Ohara T,et al.Effective control of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis with immunochemotherapy[J].Blood,1999,93(6):1869-1874.
18.	Okamura T,Hatsukawa Y,Arai H,et al.Blood stem-cell transplantation for chronic active Epstein-Barr virus with lymphoproliferation[J].Lancet,2000,356(9225):223-224.
19.	Uehara T,Nakaseko C,Hara S,et al.Successful control of Epstein-Barr virus (EBV)-infected cells by allogeneic nonmyeloablative stem cell transplantation in a patient with the lethal form of chronic active EBV infection[J].Am J Hematol,2004,76(4):368-372.
20.	Okamura T,Kishimoto T,Inoue M,et al.Unrelated bone marrow transplantation for Epstein-Barr virus-associated T/NK-cell lymphoproliferative disease[J].Bone Marrow Transplant,2003,31(2):105-111.
21.	Miyamura T,Chayama K,Wada T,et al.Two cases of chronic active Epstein-Barr virus infection in which EBV-specific cytotoxic T lymphocyte was induced after allogeneic bone marrow transplantation[J].Pediatr Transplant,2008,12(5):588-592.

1. Okano M,Kawa K,Kimura H,et al.Proposed guidelines for diagnosing chronic active Epstein-Barr virus infection[J].Am J Hematol,2005,80(1):64-69.
2. Kimura H,Hoshino Y,Kanegane H,et al.Clinical and virologic characteristics of chronic active Epstein-Barr virus infection[J].Blood,2001,98(2):280-286.
3. Kimura H,Morishima T,Kanegane H,et al.Prognostic factors for chronic active Epstein-Barr virus infection[J].J Infect Dis,2003,187(4):527-533.
4. 洪亮,金嘉琳,張文宏,等.成人慢性活動性EB病毒感染三例分析[J].中華臨床感染病雜志,2012,5(2):101-103.
5. 段紅梅,謝正德,申昆玲.慢性活動性EB病毒感染的研究進展[J].臨床兒科雜志,2007,25(2):153-156.
6. Yamashita N,Kimura H,Morishima T.Virological aspects of Epstein-Barr virus infections[J].Acta Med Okayama,2005,59(6):239-246.
7. 宋紅梅,吳曉燕,王薇,等.嚴重慢性活動性EB病毒感染12例臨床回顧與隨訪[J].中華兒科雜志,2009,47(9):682-686.
8. Okano M.Overview and problematic standpoints of severe chronic active Epstein-Barr virus infection syndrome[J].Crit Rev Oncol Hematol,2002,44(3):273-282.
9. Cohen JI.Optimal treatment for chronic active Epstein-Barr virus diseases[J].Pediatr Transplant,2009,13(4):393-396.
10. Gulley ML,Glaser SL,Craig FE,et al.Guidelines for interpreting EBER in situ hybridization and LMP1 immunohistochemical tests for detecting Epstein-Barr virus in Hodgkin lymphoma[J].Am J Clin Pathol,2002,117(2):259-267.
11. de Boer M,Mol MJ,Bogman MJ,et al.Chronic active Epstein-Barr virus infection in an adult with no detectable immune deficiency[J].Neth J Med,2003,61(11):376-382.
12. Gershburg E,Pagano JS.Epstein-Barr virus infections:prospects for treatment[J].J Antimicrob Chemother,2005,56(2):277-281.
13. Sakai Y,Ohga S,Tonegawa Y,et al.Interferon-alpha therapy for chronic active Epstein-Barr virus infection:potential effect on the development of T-lymphoproliferative disease[J].J Pediatr Hematol Oncol,1998,20(4):342-346.
14. Fujisaki T,Nagafuchi S,Okamura T.Gamma-interferon for severe chronic active Epatein-Barr virus[J].Ann Intern Med,1993,118(6):474-475.
15. Kawa-Ha K,Franco E,Doi S,et al.Successful treatment of chronic active Epstein-Barr virus infection with recombinant interleukin-2[J].Lancet,1987,1(8525):154.
16. Kimura H.Pathogenesis of chronic active Epstein-Barr virus infection:Is this an infectious disease,lymphoproliferative disorder,or immunodeficiency [J].Rev Med Virol,2006,16(4):251-261.
17. Imashuku S,Hibi S,Ohara T,et al.Effective control of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis with immunochemotherapy[J].Blood,1999,93(6):1869-1874.
18. Okamura T,Hatsukawa Y,Arai H,et al.Blood stem-cell transplantation for chronic active Epstein-Barr virus with lymphoproliferation[J].Lancet,2000,356(9225):223-224.
19. Uehara T,Nakaseko C,Hara S,et al.Successful control of Epstein-Barr virus (EBV)-infected cells by allogeneic nonmyeloablative stem cell transplantation in a patient with the lethal form of chronic active EBV infection[J].Am J Hematol,2004,76(4):368-372.
20. Okamura T,Kishimoto T,Inoue M,et al.Unrelated bone marrow transplantation for Epstein-Barr virus-associated T/NK-cell lymphoproliferative disease[J].Bone Marrow Transplant,2003,31(2):105-111.
21. Miyamura T,Chayama K,Wada T,et al.Two cases of chronic active Epstein-Barr virus infection in which EBV-specific cytotoxic T lymphocyte was induced after allogeneic bone marrow transplantation[J].Pediatr Transplant,2008,12(5):588-592.

West China Medical Journal

Clinical Analysis of Chronic Active Epstein-Barr Virus Infection

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