Abstract
We describe the case of a 23-month-old male infant with Epstein-Barr virus (EBV)-associated lymphoproliferative disorder, which mimicked the recurrence of EBV-associated hemophagocytic lymphohistiocytosis. Chemotherapy with dexamethasone, etoposide, and cyclosporine resolved fever, hepatosplenomegaly, and pancytopenia. However, on day 81 of illness, the patient developed similar symptoms. Plasma EBV-DNA levels markedly increased again, but no T-cell clonality was observed. B cells were identified to be infected with EBV. He was successfully treated with rituximab, dexamethasone and etoposide. When recurrence of EBV-associated hemophagocytic lymphohistiocytosis is suspected, performing tests to identify the infected cells will enable accurate understanding of the clinical condition, resulting in proper treatments.
Publication types
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Case Reports
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Research Support, Non-U.S. Gov't
MeSH terms
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Antineoplastic Combined Chemotherapy Protocols / administration & dosage*
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B-Lymphocytes* / metabolism
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B-Lymphocytes* / virology
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DNA, Viral / blood*
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Dexamethasone / administration & dosage
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Epstein-Barr Virus Infections* / blood
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Epstein-Barr Virus Infections* / diagnosis
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Epstein-Barr Virus Infections* / drug therapy
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Etoposide / administration & dosage
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Herpesvirus 4, Human*
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Humans
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Infant
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Lymphohistiocytosis, Hemophagocytic* / blood
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Lymphohistiocytosis, Hemophagocytic* / diagnosis
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Lymphohistiocytosis, Hemophagocytic* / drug therapy
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Lymphohistiocytosis, Hemophagocytic* / virology
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Lymphoproliferative Disorders* / blood
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Lymphoproliferative Disorders* / diagnosis
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Lymphoproliferative Disorders* / drug therapy
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Lymphoproliferative Disorders* / virology
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Male
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Rituximab / administration & dosage
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T-Lymphocytes / metabolism
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T-Lymphocytes / virology
Substances
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DNA, Viral
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Rituximab
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Etoposide
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Dexamethasone