title: Aggressive EBV-associated lymphoproliferative disorder: a prodrome to diffuse large B-cell lymphoma?
abstract: A 19-year-old male patient presented with intermittent high fever and left cervical lymphadenopathy. The lymph node biopsy findings were interpreted as "Epstein-Barr virus (EBV)-associated lymphoproliferative disorder consistent with infectious mononucleosis." No molecular studies were performed at that time. The patient was followed without treatment. Five months later, the patient again presented with fever, lymphadenopathy, and splenomegaly. The lymph node biopsy showed features of a diffuse large B-cell lymphoma. Molecular studies on this lymph node biopsy showed a clonal EBV population, although polymerase chain reaction studies failed to reveal a clonal B-cell or T-cell population. A concurrent bone marrow biopsy showed features consistent with hemophagocytic syndrome. He had elevated ferritin, soluble interleukin-2 receptors and persistent EBV viremia. The patient responded to Rituxan for a short period with undetectable EBV levels. Subsequent right cervical lymph node, liver, and jejunal biopsies showed involvement by diffuse large B-cell lymphoma and the patient expired soon thereafter.
authors.affiliation: Department of Pathology and Internal Medicine, Stanford University School of Medicine, Stanford, CA 94305-5324, USA.
authors.forename: Rashmi
authors.initials: R
authors.lastname: Batra
authors.forename: Bruno C
authors.initials: BC
authors.lastname: Medeiros
authors.forename: James L
authors.initials: JL
authors.lastname: Zehnder
authors.forename: Roger A
authors.initials: RA
authors.lastname: Warnke
authors.forename: Yasodha
authors.initials: Y
authors.lastname: Natkunam
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage / Q000627:therapeutic use; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000188:drug therapy / Q000473:pathology* / Q000821:virology; D017809:Fatal Outcome; D005293:Ferritins / Q000097:blood; D006801:Humans; D007244:Infectious Mononucleosis / Q000150:complications / Q000188:drug therapy / Q000473:pathology* / Q000821:virology; D008198:Lymph Nodes / Q000473:pathology / Q000821:virology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications / Q000188:drug therapy / Q000473:pathology* / Q000821:virology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000150:complications / Q000188:drug therapy / Q000473:pathology* / Q000821:virology; D008297:Male; D015375:Receptors, Interleukin-2 / Q000097:blood; D000069283:Rituximab; D019562:Viral Load / Q000187:drug effects; D055815:Young Adult
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D015375:Receptors, Interleukin-2; D000069283:Rituximab; D005293:Ferritins