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15 May 2008

Volume 46, Number 10
Clinical Infectious Diseases 2008;46:1525–1534
© 2008 by the Infectious Diseases Society of America. All rights reserved.
1058-4838/2008/4610-0007$15.00
MAJOR ARTICLE

Clinical and Virological Characteristics of 15 Patients with Chronic Active Epstein‐Barr Virus Infection Treated with Hematopoietic Stem Cell Transplantation

Kensei Gotoh,1

Yoshinori Ito,1

Yukiko Shibata‐Watanabe,1

Jun‐ichi Kawada,1

Yoshiyuki Takahashi,1

Hiroshi Yagasaki,1

Seiji Kojima,1

Yukihiro Nishiyama,2 and

Hiroshi Kimura2

Departments of 1Pediatrics and 2Virology, Nagoya University Graduate School of Medicine, Nagoya, Japan

Background.Chronic active Epstein‐Barr virus (EBV) infection is characterized by recurrent infectious mononucleosis–like symptoms, and infected patients have high viral loads in their peripheral blood. Standard therapy for the disease has not yet been established. Recently, hematopoietic stem cell transplantation (HSCT) has been introduced and has the potential to become a standard treatment, although guidelines for HSCT to treat chronic active EBV infection have not yet been proposed.

Methods.Fifteen patients were retrospectively analyzed, both clinically and virologically, to investigate the factors associated with prognosis of chronic active EBV infection treated with HSCT.

Results.After HSCT, 7 patients died after survival periods that ranged from 1 to 16 months (mean duration of survival after HSCT, 5 months). Three patients were considered to have died of transplantation‐related complications. The duration between infection onset and diagnosis was significantly longer in patients who died than in those who survived. Five of the 7 patients who died experienced 3 life‐threatening complications. The plasma concentrations of interferon‐γ, interleukin‐10, thrombomodulin, and soluble E‐selectin did not differ significantly between the groups of patients. With regard to sequence variations in the EBV latent membrane protein 1 gene, no specific patterns were found in the patients who died. Importantly, the plasma EBV load at diagnosis was significantly higher in patients who died than in living patients. Moreover, plasma viral load was shown to be an important factor to monitor during follow‐up for patients after HSCT.

Conclusions.The number of life‐threatening complications and plasma viral load are indicative of the stage of disease progression and may be useful factors for predicting the outcome of HSCT.

Received 16 September 2007; accepted 20 December 2007; electronically published 4 April 2008.

  • (See the editorial commentary by Gottschalk on pages 1535–6)

Reprints or correspondence: Dr. Hiroshi Kimura, Dept. of Virology, Nagoya University Graduate School of Medicine, 65 Tsurumai‐cho, Showa‐ku, Nagoya 466‐8550, Japan ().

Cited by

J. I. Cohen, H. Kimura, S. Nakamura, Y.-H. Ko, E. S. Jaffe. (2009) Epstein-Barr virus-associated lymphoproliferative disease in non-immunocompromised hosts: a status report and summary of an international meeting, 8-9 September 2008. Annals of Oncology
Online publication date: 10-Jul-2009.
CrossRef
Jeffrey I. Cohen. (2008) Optimal treatment for chronic active Epstein-Barr virus disease. Pediatric Transplantation
Online publication date: 1-Dec-2008.
CrossRef
Hiroshi Kimura, Yoshinori Ito, Ritsuro Suzuki, Yukihiro Nishiyama. (2008) Measuring Epstein-Barr virus (EBV) load: the significance and application for each EBV-associated disease. Reviews in Medical Virology 18:5, 305-319
Online publication date: 1-Oct-2008.
CrossRef
Stephen Gottschalk. (2008) Editorial Commentary: Clinical and Virological Characteristics of Patients with Chronic Active Epstein‐Barr Virus Infection Treated with Hematopoietic Stem Cell Transplantation: Insights and Questions. Clinical Infectious Diseases 46:10, 1535-1536
Online publication date: 15-May-2008.
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