EBV Infection in XLP1 Manifested Solely by Behavioral Aggression and Effective Treatment Using Rituximab

Patients with X-linked lymphoproliferative disease 1 (XLP1) are exquisitely susceptible to Epstein-Barr virus (EBV), with the first EBV infection often resulting in rapid death. In a manner not previously described, a 5-year-old patient with XLP1 presented solely with behavioral aggression, with no...

Full description

Saved in:
Bibliographic Details
Published in:Case reports in immunology 2018-01, Vol.2018 (2018), p.1-4
Main Authors: Korah-Sedgwick, Michelle M., Wall, Luke A.
Format: Article
Language:English
Subjects:
Aggression
Antigens
Case Report
Case reports
Cerebrospinal fluid
Cytotoxicity
Disease
Epstein-Barr virus
Frontal lobe
Ganciclovir
Hematopoietic stem cells
Immunoglobulin M
Immunotherapy
Infections
Lymphocytes
Lymphoma
Magnetic resonance imaging
Monoclonal antibodies
Mutation
Patients
Rituximab
Stem cells
Targeted cancer therapy
Transplants & implants
Viremia
Citations: Items that this one cites
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Patients with X-linked lymphoproliferative disease 1 (XLP1) are exquisitely susceptible to Epstein-Barr virus (EBV), with the first EBV infection often resulting in rapid death. In a manner not previously described, a 5-year-old patient with XLP1 presented solely with behavioral aggression, with no laboratory evidence of organ dysfunction or inflammation. Although EBV-IgM was negative, PCR confirmed the presence of EBV in both the blood and cerebrospinal fluid. MRI of the brain showed frontal lobe foci. After failure to eradicate his viremia with ganciclovir, rituximab was administered. EBV was eradicated from the blood after the second rituximab infusion and remained absent for 5 months, at which time he underwent hematopoietic stem cell transplant. Although EBV classically produces fulminant infection in patients with XLP1, this case demonstrates that EBV infection may be initially subtle. Acute change in behavior should prompt evaluation. This case also demonstrates the possible effectiveness of rituximab in the treatment of acute EBV infection.
ISSN:2090-6609
2090-6617
DOI:10.1155/2018/3705376