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.
journal_name
Bloodjournal_title
Bloodauthors
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,Stdoi
10.1182/blood-2010-11-316745subject
Has Abstractpub_date
2011-06-02 00:00:00pages
5835-49issue
22eissn
0006-4971issn
1528-0020pii
blood-2010-11-316745journal_volume
117pub_type
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