Characterization and treatment of chronic active Epstein-Barr virus disease

A 28-year experience in the United States

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 & 2 others Wyndham Wilson, Stephen E. Straus

Research output: Contribution to journalArticle

137 Citations (Scopus)

Abstract

Chronic active EBV disease (CAEBV) is a lymphoproliferative disorder characterized by markedly elevated levels of antibody to EBV or EBV DNA in the blood and EBV RNA or protein in lymphocytes in tissues. We present our experience with CAEBV during the last 28 years, including the first 8 cases treated with hematopoietic stem cell transplantation in the United States. Most cases of CAEBV have been reported from Japan. Unlike CAEBV in Japan, where EBV is nearly always found in T or natural killer (NK) cells in tissues, EBV was usually detected in B cells in tissues from our patients. Most patients presented with lymphadenopathy and splenomegaly; fever, hepatitis, and pancytopenia were common. Most patients died of infection or progressive lymphoproliferation. Unlike cases reported from Japan, our patients often showed a progressive loss of B cells and hypogammaglobulinemia. Although patients with CAEBV from Japan have normal or increased numbers of NK cells, many of our patients had reduced NK-cell numbers. Although immunosuppressive agents, rituximab, autologous cytotoxic T cells, or cytotoxic chemotherapy often resulted in short-term remissions, they were not curative. Hematopoietic stem cell transplantation was often curative for CAEBV, even in patients with active lymphoproliferative disease that was unresponsive to chemotherapy. These studies are registered at http://www.clinicaltrials.gov as NCT00032513 for CAEBV, NCT00062868 and NCT00058812 for EBV-specific T-cell studies, and NCT00578539 for the hematopoietic stem cell transplantation protocol.

Original languageEnglish (US)
Pages (from-to)5835-5849
Number of pages15
JournalBlood
Volume117
Issue number22
DOIs
StatePublished - Jun 2 2011
Externally publishedYes

Fingerprint

Virus Diseases
Human Herpesvirus 4
Viruses
Stem cells
Chemotherapy
T-cells
Therapeutics
Tissue
Hematopoietic Stem Cell Transplantation
Japan
Cells
Natural Killer Cells
Lymphocytes
Immunosuppressive Agents
B-Lymphocytes
T-Lymphocytes
Blood
Drug Therapy
Agammaglobulinemia
Pancytopenia

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

Cite this

Cohen, J. I., Jaffe, E. S., Dale, J. K., Pittaluga, S., Heslop, H. E., Rooney, C. M., ... Straus, S. E. (2011). Characterization and treatment of chronic active Epstein-Barr virus disease: A 28-year experience in the United States. Blood, 117(22), 5835-5849. https://doi.org/10.1182/blood-2010-11-316745

Characterization and treatment of chronic active Epstein-Barr virus disease : A 28-year experience in the United States. / Cohen, Jeffrey I.; Jaffe, Elaine S.; Dale, Janet K.; Pittaluga, Stefania; Heslop, Helen E.; Rooney, Cliona M.; Gottschalk, Stephen; Bollard, Catherine M.; Rao, V. Koneti; Marques, Adriana; Burbelo, Peter D.; Turk, Siu Ping; Fulton, Rachael; Wayne, Alan S.; Little, Richard F.; Cairo, Mitchell S.; El-Mallawany, Nader K.; Fowler, Daniel; Sportes, Claude; Bishop, Michael R.; Wilson, Wyndham; Straus, Stephen E.

In: Blood, Vol. 117, No. 22, 02.06.2011, p. 5835-5849.

Research output: Contribution to journalArticle

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 2011, 'Characterization and treatment of chronic active Epstein-Barr virus disease: A 28-year experience in the United States', Blood, vol. 117, no. 22, pp. 5835-5849. https://doi.org/10.1182/blood-2010-11-316745
Cohen, Jeffrey I. ; Jaffe, Elaine S. ; Dale, Janet K. ; Pittaluga, Stefania ; Heslop, Helen E. ; Rooney, Cliona M. ; Gottschalk, Stephen ; Bollard, Catherine M. ; Rao, V. Koneti ; Marques, Adriana ; Burbelo, Peter D. ; Turk, Siu Ping ; Fulton, Rachael ; Wayne, Alan S. ; Little, Richard F. ; Cairo, Mitchell S. ; El-Mallawany, Nader K. ; Fowler, Daniel ; Sportes, Claude ; Bishop, Michael R. ; Wilson, Wyndham ; Straus, Stephen E. / Characterization and treatment of chronic active Epstein-Barr virus disease : A 28-year experience in the United States. In: Blood. 2011 ; Vol. 117, No. 22. pp. 5835-5849.
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