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

2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
Michelle M. Korah-Sedgwick ◽  
Luke A. Wall
Keyword(s):  
Epstein Barr Virus ◽  
Hematopoietic Stem Cell Transplant ◽  
Lymphoproliferative Disease ◽  
Cell Transplant ◽  
Hematopoietic Stem ◽  
Barr Virus ◽  
Ebv Infection ◽  
Epstein Barr ◽  
Behavioral Aggression ◽  
The Brain

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.

scholarly journals Factors Associated with Post-Transplant Active Epstein-Barr Virus Infection and Lymphoproliferative Disease in Hematopoietic Stem Cell Transplant Recipients: A Systematic Review and Meta-Analysis

Vaccines ◽  
2021 ◽  
Vol 9 (3) ◽  
pp. 288
Author(s):  
Pascal Roland Enok Bonong ◽  
Monica Zahreddine ◽  
Chantal Buteau ◽  
Michel Duval ◽  
Louise Laporte ◽  
...  
Keyword(s):  
Risk Factors ◽  
Systematic Review ◽  
Epstein Barr Virus ◽  
Lymphoproliferative Disease ◽  
Hematopoietic Stem ◽  
Post Transplant ◽  
Barr Virus ◽  
Ebv Infection ◽  
Relative Risks ◽  
Epstein Barr

This systematic review was undertaken to identify risk factors associated with post-transplant Epstein–Barr virus (EBV) active infection and post-transplant lymphoproliferative disease (PTLD) in pediatric and adult recipients of hematopoietic stem cell transplants (HSCT). A literature search was conducted in PubMed and EMBASE to identify studies published until 30 June 2020. Descriptive information was extracted for each individual study, and data were compiled for individual risk factors, including, when possible, relative risks with 95% confidence intervals and/or p-values. Meta-analyses were planned when possible. The methodological quality and potential for bias of included studies were also evaluated. Of the 3362 titles retrieved, 77 were included (62 for EBV infection and 22 for PTLD). The overall quality of the studies was strong. Several risk factors were explored in these studies, but few statistically significant associations were identified. The use of anti-thymocyte globulin (ATG) was identified as the most important risk factor positively associated with post-transplant active EBV infection and with PTLD. The pooled relative risks obtained using the random-effect model were 5.26 (95% CI: 2.92–9.45) and 4.17 (95% CI: 2.61–6.68) for the association between ATG and post-transplant EBV infection and PTLD, respectively. Other risk factors for EBV and PTLD were found in the included studies, such as graft-versus-host disease, type of conditioning regimen or type of donor, but results are conflicting. In conclusion, the results of this systematic review indicate that ATG increases the risk of EBV infection and PTLD, but the link with all other factors is either nonexistent or much less convincing.

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