Epstein-Barr Virus (EBV)-induced B-cell Lymphoproliferative Disorder Mimicking the Recurrence of EBV-associated Hemophagocytic Lymphohistiocytosis.

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.

• Authors: Yuki Yatsushiro, Takuro Nishikawa, Aki Saito, Yozo Nakazawa, Ken-Ichi Imadome, Shunsuke Nakagawa, Yuichi Kodama, Yasuhiro Okamoto, Hirokazu Kanegane, Yoshifumi Kawano
• Journal: Journal of pediatric hematology/oncology (J Pediatr Hematol Oncol) Vol. 41 Issue 1 Pg. e44-e46 (01 2019) ISSN: 1536-3678 [Electronic] United States
• PMID: 29324572 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• DNA, Viral
• Rituximab
• Etoposide
• Dexamethasone

Topics

• Antineoplastic Combined Chemotherapy Protocols (administration & dosage)
• B-Lymphocytes (metabolism, virology)
• DNA, Viral (blood)
• Dexamethasone (administration & dosage)
• Epstein-Barr Virus Infections (blood, diagnosis, drug therapy)
• Etoposide (administration & dosage)
• Herpesvirus 4, Human
• Humans
• Infant
• Lymphohistiocytosis, Hemophagocytic (blood, diagnosis, drug therapy, virology)
• Lymphoproliferative Disorders (blood, diagnosis, drug therapy, virology)
• Male
• Rituximab (administration & dosage)
• T-Lymphocytes (metabolism, virology)