Nihon Bika Gakkai Kaishi (Japanese Journal of Rhinology)
Online ISSN : 1883-7077
Print ISSN : 0910-9153
ISSN-L : 0910-9153
Original Articles
Epstein-Barr Virus-related Lymphoproliferative Disorder Associated with X-linked Lymphoproliferative Syndrome: A Case Report
Ken IwanagaKazuhiko MinamiTomoyuki Haji
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JOURNAL FREE ACCESS

2013 Volume 52 Issue 1 Pages 43-48

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Abstract

X-linked lymphoproliferative syndrome (XLP) is a congenital immunodeficiency which involves a defective specific immune response to Epstein-Barr virus (EBV).
We report a case of EBV-related lymphoproliferative disorder associated with an XLP primary lesion in the nasal sinus.
A 3-year, 9-month-old boy was being followed as an outpatient on suspicion of EBV infection, and onset of EBV encephalitis led to admission to the Paediatrics Department. He was started on multiagent chemotherapy, however this was not effective, leading to a congenital immunodeficiency being suspected. XLP was diagnosed using a genetic test 61 days after admission. On day 95, a brain MRI revealed a solid lesion spanning from the right nasal cavity to the maxillary sinus. Hemorrhage from the same lesion led to a referral to our department on day 125. A pale red hemorrhagic mass was observed in the right nasal cavity, and biopsy allowed a diagnosis of CD20+ EBV-related lymphoproliferative disorder. On day 143, rituximab was started, but on day 151, the patient died due to worsening systemic condition.
XLP is a disease with a poor prognosis. Over 70% of patients die by the age of 10 due to fatal infectious mononucleosis. Malignant lymphoma was associated in 30% of XLP cases, however, a literature search revealed no reports involving primary lesions of the nasal sinus.
Reports describe good results for hematopoietic stem cell transplantation, with this becoming the main focus for the treatment of XLP.

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© 2013 Japan Rhinologic Society
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