title: Primary immunodeficiencies associated with EBV-Induced lymphoproliferative disorders.
abstract: Primary immunodeficiency diseases (PIDs) are a subgroup of inherited immunological disorders that increase susceptibility to viral infections. Among the range of viral pathogens involved, EBV remains a major threat because of its high prevalence of infection among the adult population and its tendency to progress to life-threatening lymphoproliferative disorders (LPDs) and/or malignancy. The high mortality in immunodeficient patients with EBV-driven LPDs, despite institution of diverse and often intensive treatments, prompts the need to better study these PIDs to identify and understand the affected molecular pathways that increase susceptibility to EBV infection and progression. In this article, we have provided a detailed literature review of the reported cases of EBV-driven LPDs in patients with PID. We discuss the PIDs associated with development of EBV-LPDs. Then, we review the nature and the therapeutic outcome of common EBV- driven LPDs in the PID patients and review the mechanisms common to the major PIDs. Deep study of these common pathways and gaining a better insight into the disease nature and outcomes, may lead to earlier diagnosis of the disease, choosing the best treatment modalities available and development of novel therapeutic strategies to decrease morbidity and mortality brought about by EBV infection.
authors.affiliation: Research Center for Immunodeficiencies, Children's Medical School, Tehran University of Medical Sciences, Tehran, Iran; Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran; International Hematology/Oncology Of Pediatrics Experts (IHOPE), Universal Scientific Education and Research Network (USERN), Tehran, Iran.
authors.forename: Mahsima
authors.initials: M
authors.lastname: Shabani
authors.affiliation: Department of Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA.
authors.forename: Kim E
authors.initials: KE
authors.lastname: Nichols
authors.affiliation: Research Center for Immunodeficiencies, Children's Medical School, Tehran University of Medical Sciences, Tehran, Iran; Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran; Systematic Review and Meta-Analysis Expert Group (SRMEG), Universal Scientific Education and Research Network (USERN), Boston, MA, USA. Electronic address: Rezaei_nima@tums.ac.ir.
authors.forename: Nima
authors.initials: N
authors.lastname: Rezaei
mesh_terms: D000818:Animals; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D004854:Herpesvirus 4, Human; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000276:immunology*; D008232:Lymphoproliferative Disorders / Q000276:immunology*; D009154:Mutation
keywords: Epstein-Barr virus; Lymphoproliferative disorders; Primary immunodeficiency diseases