Dual Threat of Epstein-Barr Virus: an Autopsy Case Report of HIV-Positive Plasmablastic Lymphoma Complicating EBV-Associated Hemophagocytic Lymphohistiocytosis.
Epstein-Barr virus (EBV) reactivation causes serious diseases in immunocompromised hosts, such as acquired immunodeficiency syndrome (AIDS). We report on a case of plasmablastic lymphoma (PBL) with hemophagocytic lymphohistiocytosis (HLH).A-53-year-old Japanese man was diagnosed with PBL and AIDS. In addition to combined antiretroviral therapy, HyperCVAD (cyclophosphamide, doxorubicin, vincristine, prednisone)/high-dose methotrexate + cytarabine was initiated immediately. Partial remission was attained with chemotherapy. However, the patient developed HLH and died despite intensive therapy. Autopsy findings suggested that PBL was controlled, and immunosuppression appeared to cause fatal infection. The patient showed high titers of EBV viral-capsid antigen (VCA)-IgG (1:2560) on PBL diagnosis and high EBV-DNA levels throughout the clinical course. Moreover, EBV-DNA was detected in the fraction of CD8-positive cells, which strongly supports the pathogenesis of EBV-associated HLH.Our report highlights the importance of EBV control in patients with EBV-positive AIDS lymphoma. EBV not only behaves as the etiologic pathogen of PBL but also can be a trigger of HLH, the fatal complication. Careful follow-up of the EBV status should be performed, and if needed, preemptive anti-EBV therapy should also be considered to prevent EBV-associated complications such as HLH.
['Koizumi|Yusuke|Y|0000-0001-9369-8767', 'Imadome|Ken-Ichi|KI|', 'Ota|Yasunori|Y|', 'Minamiguchi|Hitoshi|H|', 'Kodama|Yoshinori|Y|', 'Watanabe|Dai|D|', 'Mikamo|Hiroshige|H|', 'Uehira|Tomoko|T|', 'Okada|Seiji|S|', 'Shirasaka|Takuma|T|']
D001344:Autopsy D015415:Biomarkers D060085:Coinfection D020031:Epstein-Barr Virus Infections D015658:HIV Infections D004854:Herpesvirus 4, Human D006801:Humans D007150:Immunohistochemistry D016130:Immunophenotyping D051359:Lymphohistiocytosis, Hemophagocytic D008297:Male D008875:Middle Aged D000069293:Plasmablastic Lymphoma D000072078:Positron Emission Tomography Computed Tomography
Epstein-Barr virus (EBV) acquired immunodeficiency syndrome (AIDS) plasmablastic lymphoma (PBL) hemophagocytic lymphohistiocytosis (HLH) hypercytokinemia