Chronic active Epstein-Barr virus 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. [Lymphoproliferative means producing excessive amounts of lymphocytes; typical of immune dysfunction or deficiency.]
We present our experience with CAEBV over the last 28 years including the first 8 cases treated with hematopoietic stem cell transplantation (HSCT) in the U.S. [HSCT are stem cells able to give rise to all blood cell types including those responsible for cell-mediated immunity.]
Most cases of CAEBV have been reported from Japan. Unlike CAEBV in Japan where EBV is nearly always found in T or NK cells in tissues, EBV was usually detected in B cells in tissues from our patients. [Different strains?]
Most patients presented with lymphadenopathy and splenomegaly; fever, hepatitis, and pancytopenia were common. Most died of infection or progressive lymphoproliferation.
Unlike cases reported from Japan, our patients often showed a progressive loss of B cells, and hypogammaglobulinemia.
While 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.
HSCT was often curative for CAEBV, even in patients with active lymphoproliferative disease that was unresponsive to chemotherapy.
These studies are registered at www.clinicaltrials.gov as NCT00032513 for CAEBV, NCT00062868 and NCT00058812 for EBV-specific T cell studies, and NCT00578539 for the hematopoietic stem cell transplant protocol.
Source: Blood, Mar 31, 2011. PMID: 21454450, by 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. Laboratory of Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland, USA.