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Free Content A boy with fever, lymphadenopathy, hepatosplenomegaly, and lymphocytosis

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Proliferation of the lymphoid system should arouse suspicion of a potentially serious illness. We present a 4.5-year-old boy who developed fever, vomiting, diarrhea, lymphadenopathy, hepatosplenomegaly, lymphocytosis, anemia, thrombocytopenia, and increased liver enzymes. Lymph node and bone marrow biopsies showed lymphoproliferation, Epstein-Barr virus (EBV) infection, and hemophagocytosis leading to the diagnosis of hemophagocytic lymphohistiocytosis (HLH). Chemotherapy was initiated for HLH with dexamethasone, etoposide, and cyclosporine. Because of a high level of EBV viremia, rituximab was added a few days later and resulted in a remarkable drop in the EBV in the circulation but not in the cerebrospinal fluid. However, the patient succumbed to encephalitis, pneumonia, and cardiopulmonary failure. Autopsy revealed the presence of EBV in the brain, indicating the ineffectiveness of rituximab therapy in treating central nervous system infection with EBV.

Keywords: EBV; hemophagocytosis; hepatosplenomegaly; lymphadenopathy; lymphocytosis; lymphoproliferative disease; rituximab

Document Type: Research Article


Affiliations: 1: Section of Allergy and Immunology, Louisiana State University Health Sciences Center, Shreveport, Louisiana 2: Section of Hematology and Oncology, Louisiana State University Health Sciences Center, Shreveport, Louisiana 3: Infectous Disease Section, Department of Pediatrics, Louisiana State University Health Sciences Center, Shreveport, Louisiana 4: Department of Pathology, Louisiana State University Health Sciences Center, Shreveport, Louisiana

Publication date: March 1, 2008

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  • Allergy and Asthma Proceedings is a peer reviewed publication dedicated to distributing timely scientific research regarding advancements in the knowledge and practice of allergy, asthma and immunology. Its primary readership consists of allergists and pulmonologists.

    The goal of the Proceedings is to publish articles with a predominantly clinical focus which directly impact quality of care for patients with allergic disease and asthma.

    Featured topics include asthma, rhinitis, sinusitis, food allergies, allergic skin diseases, diagnostic techniques, allergens, and treatment modalities. Published material includes peer-reviewed original research, clinical trials and review articles.

    Articles marked "F" offer free full text for personal noncommercial use only.

    The journal is indexed in Thomson Reuters Web of Science and Science Citation Index Expanded, plus the National Library of Medicine's PubMed service.
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