title: An Epstein-Barr virus-positive diffuse large B-cell lymphoma presenting as multi-organ failure: a catastrophic lymphomatosis with fulminant visceral organ dissemination resulting in a precipitous death in a 59-year-old female with no identifiable etiology for immunodeficiency.
abstract: Epstein-Barr virus (EBV)-positive diffuse large B-cell lymphoma (EBV+ DLBCL) of the elderly is an aggressive B-cell neoplasm related to age-associated impaired immunity. We report such a case in a 59-year-old woman with a catastrophic disease course. The patient initially presented with fever, fatigue, malaise and weakness over one-week period. Despite empirical treatment with antibiotics and antiviral agents, she subsequently developed multi-organ failure and coagulopathy. Radiographic imaging revealed hepatomegaly, splenomegaly, pleural effusion, and ascites. Her complete blood cell count showed marked leukocytosis, anemia and thrombocytopenia. Morphologic examination of blood smear demonstrated many abnormal plasmacytoid lymphocytes, and flow cytometric analysis detected an intermediate-large mature B-cell population (69%) without detectable surface immunoglobulin. High copy numbers of EBV genome were detected in the blood by PCR. A diagnosis of EBV+ DLBCL, leukemic phase, was made. Despite aggressive treatment and supportive care, the patient succumbed to multi-organ failure one week after initial presentation. Autopsy demonstrated EBV+ DLBCL infiltration in all the organs examined. This case describes an unusual presentation of EBV+ DLBCL and highlights the necessity of pertinent ancillary tests to avoid delay in the diagnosis and treatment.
authors.affiliation: Department of Pathology, Duke University Medical Center, Durham, NC 27710, United States. Electronic address: endi.wang@duke.edu.
authors.forename: Endi
authors.initials: E
authors.lastname: Wang
authors.affiliation: Department of Pathology, Duke University Medical Center, Durham, NC 27710, United States.
authors.forename: Paulie
authors.initials: P
authors.lastname: Papavassiliou
authors.affiliation: Department of Pathology, Duke University Medical Center, Durham, NC 27710, United States.
authors.forename: Qiang
authors.initials: Q
authors.lastname: Wei
authors.affiliation: Department of Pathology, Duke University Medical Center, Durham, NC 27710, United States.
authors.forename: M Quinn
authors.initials: MQ
authors.lastname: Wickham
authors.affiliation: Department of Pathology, Duke University Medical Center, Durham, NC 27710, United States.
authors.forename: Lisa
authors.initials: L
authors.lastname: Cichon
authors.affiliation: Department of Pathology, Duke University Medical Center, Durham, NC 27710, United States.
authors.forename: Alan D
authors.initials: AD
authors.lastname: Proia
mesh_terms: D020031:Epstein-Barr Virus Infections / Q000150:complications*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D016403:Lymphoma, Large B-Cell, Diffuse / Q000473:pathology* / Q000503:physiopathology / Q000821:virology*; D008875:Middle Aged; D009102:Multiple Organ Failure / Q000209:etiology*
keywords: Diffuse large B-cell lymphoma; Epstein–Barr virus; Multi-organ failure; Precipitous death; Visceral dissemination