scholarly journals Primary and Acquired Immunodeficiencies Associated With Severe Varicella-Zoster Virus Infections

2020 ◽  
Author(s):  
Rahila Ansari ◽  
Lindsey B Rosen ◽  
Andrea Lisco ◽  
Don Gilden ◽  
Steven M Holland ◽  
...  
Keyword(s):  
Varicella Zoster Virus ◽  
Mononuclear Cells ◽  
Critical Role ◽  
Virus Infections ◽  
Peripheral Blood Mononuclear ◽  
Varicella Zoster ◽  
Cytoplasmic Dna ◽  
Immune Deficiencies ◽  
Life Threatening ◽  
Healthy Control

Abstract Background Although most cases of varicella or zoster are self-limited, patients with certain immune deficiencies may develop severe or life-threatening disease. Methods We studied a patient with varicella-zoster virus (VZV) central nervous system (CNS) vasculopathy and as part of the evaluation, tested his plasma for antibodies to cytokines. We reviewed the literature for cases of varicella or zoster associated with primary and acquired immunodeficiencies. Results We found that a patient with VZV CNS vasculopathy had antibody that neutralized interferon (IFN)-α but not IFN-γ. The patient’s plasma blocked phosphorylation in response to stimulation with IFN-α in healthy control peripheral blood mononuclear cells. In addition to acquired immunodeficiencies like human immunodeficiency virus (HIV) or autoantibodies to IFN, variants in specific genes have been associated with severe varicella and/or zoster. Although these genes encode proteins with very different activities, many affect IFN signaling pathways, either those that sense double-stranded RNA or cytoplasmic DNA that trigger IFN production, or those involved in activation of IFN stimulated genes in response to binding of IFN with its receptor. Conclusions Immune deficiencies highlight the critical role of IFN in control of VZV infections and suggest new approaches for treatment of VZV infection in patients with certain immune deficiencies.

scholarly journals Cyclophosphamide and varicella zoster virus induced disseminated intravascular coagulopathy with fatal outcome

2017 ◽  
Vol 3 (2) ◽  
pp. 303
Author(s):  
A. S. Salunke ◽  
V. A. Belgaumkar ◽  
R. B. Chavan ◽  
S. S. Tharewal
Keyword(s):  
Varicella Zoster Virus ◽  
Fatal Outcome ◽  
Pemphigus Vulgaris ◽  
Paediatric Population ◽  
Varicella Zoster Virus Infection ◽  
Virus Infections ◽  
Disseminated Intravascular Coagulopathy ◽  
Varicella Zoster ◽  
Intravascular Coagulopathy ◽  
Life Threatening

<p>Varicella is usually a mild self-limiting disease, commonly occurring in paediatric population. However, in adults and in specific groups of patients, such as those who are immunosuppressed, varicella virus infections can be fulminant and life threatening. We are hereby reporting two cases of Pemphigus vulgaris on cyclophosphamide therapy, who succumbed due to disseminated intravascular coagulopathy (DIC) after varicella zoster virus infection. </p>

scholarly journals Varicella-zoster virus CNS vasculitis and RNA polymerase III gene mutation in identical twins

2018 ◽  
Vol 5 (6) ◽  
pp. e500 ◽  
Author(s):  
Madalina E. Carter-Timofte ◽  
Anders F. Hansen ◽  
Maibritt Mardahl ◽  
Sébastien Fribourg ◽  
Franck Rapaport ◽  
...  
Keyword(s):  
Rna Polymerase ◽  
Varicella Zoster Virus ◽  
Mononuclear Cells ◽  
Inflammatory Responses ◽  
Rna Polymerase Iii ◽  
Peripheral Blood Mononuclear ◽  
Cns Vasculitis ◽  
Varicella Zoster ◽  
Polymerase Iii ◽  
Pol Iii

ObjectiveDeficiency in the cytosolic DNA sensor RNA Polymerase III (POL III) was recently described in children with severe varicella-zoster virus (VZV) infection in the CNS or lungs. Here, we describe a pair of monozygotic female twins, who both experienced severe recurrent CNS vasculitis caused by VZV reactivation. The clinical presentation and findings included recurrent episodes of headache, dizziness, and neurologic deficits, CSF with pleocytosis and intrathecal VZV antibody production, and MRI of the brain showing ischemic lesions.MethodsWe performed whole-exome sequencing and identified a rare mutation in the POL III subunit POLR3F. Subsequently, antiviral responses in patient peripheral blood mononuclear cells (PBMCs) were examined and compared with healthy controls.ResultsThe identified R50W POLR3F mutation is predicted by bioinformatics to be damaging, and when tested in functional assays, patient PBMCs exhibited impaired antiviral and inflammatory responses to the POL III agonist poly(dA:dT) and increased viral replication compared with controls.ConclusionsAltogether, these cases add genetic and immunologic evidence to the novel association between defects in sensing of AT-rich DNA present in the VZV genome and increased susceptibility to severe manifestations of VZV infection in the CNS in humans.

Immunogenicity of the Varicella-Zoster Vaccine in Community-Dwelling Non-robust Elderly Individuals Compared to Robust Elderly Individuals: A Prospective Cohort Study

2018 ◽  
Vol 74 (8) ◽  
pp. 1225-1230 ◽  
Author(s):  
Jung-Yeon Choi ◽  
Min-gu Kang ◽  
Kyoung Un Park ◽  
Wan Beom Park ◽  
Kwang-il Kim ◽  
...  
Keyword(s):  
Varicella Zoster Virus ◽  
Mononuclear Cells ◽  
Geometric Mean ◽  
Community Dwelling ◽  
Igg Antibody ◽  
Peripheral Blood Mononuclear ◽  
Elderly Individuals ◽  
Varicella Zoster ◽  
Frailty Status ◽  
Cytokine Levels

Abstract To determine whether immunogenicity of the herpes zoster live-attenuated vaccine (ZVL) in elderly individuals is influenced by frailty status and cytokine levels, individuals aged at least 65 years were prospectively administered ZVL after their frailty status and cytokine levels were assessed. Humoral and cellular immunogenicity were assessed using enzyme-linked immunosorbent assays for the varicella-zoster virus–specific IgG antibody and varicella-zoster virus–specific enzyme-linked immunospot assays, before and 6 weeks after vaccination. We enrolled 69 participants (mean age, 74.3 years); 31 were non-robust. Thirty-seven participants had a greater than 2 geometric mean fold rise in IgG antibodies, 22 more participants had at least 10 spot-forming cells/106 peripheral blood mononuclear cells (from 35 to 57 participants), and 42 experienced a twofold geometric mean fold rise increase of spot-forming cells 6 weeks after vaccination. The geometric mean fold rises of the antibody titers were similar in the robust and non-robust groups; moreover, the baseline enzyme-linked immunospot assay geometric mean values were not significantly different before or 6 weeks after vaccination. We observed no significant correlations between baseline cytokine levels and immunogenicity. Approximately half of the community-dwelling elderly participants in this study experienced a twofold rise in antibodies and one-third more participants showed at least 10 spot-forming cells after ZVL administration, suggesting that the ZVL can boost immunity in non-robust (especially pre-frail) and robust elderly individuals.

Molecular and therapeutic aspects of varicella–zoster virus infection

2005 ◽  
Vol 7 (15) ◽  
pp. 1-24 ◽  
Author(s):  
Mark Quinlivan ◽  
Judith Breuer
Keyword(s):  
Varicella Zoster Virus ◽  
Mononuclear Cells ◽  
Small Animal ◽  
Varicella Zoster Virus Infection ◽  
Peripheral Blood Mononuclear ◽  
Live Attenuated Vaccine ◽  
Narrow Host Range ◽  
Varicella Zoster

Varicella–zoster virus (VZV) is a highly species-specific member of the Herpesviridae family. The virus exhibits multiple cell tropisms, infecting peripheral blood mononuclear cells and skin cells before establishing latency in sensory neurons. Such tropisms are essential both for primary infection, which manifests itself as chickenpox (varicella), and subsequent reactivation to cause herpes zoster (shingles). The highly cell-associated nature of the virus, coupled with its narrow host range, has resulted in the lack of an animal model that mimics its diseases in humans, thereby greatly hindering the study of events in VZV pathogenesis. Despite this, extensive studies both in vitro and in vivo in small-animal models have provided a fascinating insight into molecular events that govern VZV diseases. In addition, VZV has become the first human herpes virus for which a live attenuated vaccine has been developed.

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