%0 Journal Article
%T Epstein-Barr virus-driven lymphoproliferation in inborn errors of immunity: a diagnostic and therapeutic challenge.
%A Barman P
%A Basu S
%A Goyal T
%A Sharma S
%A Siniah S
%A Tyagi R
%A Sharma K
%A Jindal AK
%A Pilania RK
%A Vignesh P
%A Dhaliwal M
%A Suri D
%A Rawat A
%A Singh S
%J Expert Rev Clin Immunol
%V 0
%N 0
%D 2024 Jul 30
%M 39066572
%F 5.124
%R 10.1080/1744666X.2024.2386427
%X <B>UNASSIGNED: </B>Inborn errors of immunity (IEI) are a group of genetically heterogeneous disorders with a wide-ranging clinical phenotype, varying from increased predisposition to infections to dysregulation of the immune system, including autoimmune phenomena, autoinflammatory disorders, lymphoproliferation, and malignancy. Lymphoproliferative disorder (LPD) in IEI refers to the nodal or extra-nodal and persistent or recurrent clonal or non-clonal proliferation of lymphoid cells in the clinical context of an inherited immunodeficiency or immune dysregulation. The Epstein-Barr virus (EBV) plays a significant role in the etiopathogenesis of LPD in IEIs. In patients with specific IEIs, lack of immune surveillance can lead to an uninhibited proliferation of EBV-infected cells that may result in chronic active EBV infection, hemophagocytic lymphohistiocytosis, and LPD, particularly lymphomas.<BR><B>UNASSIGNED: </B>We intend to discuss the pathogenesis, diagnosis, and treatment modalities directed toward EBV-associated LPD in patients with distinct IEIs.<BR><B>UNASSIGNED: </B>EBV-driven lymphoproliferation in IEIs presents a diagnostic and therapeutic problem that necessitates a comprehensive understanding of host-pathogen interactions, immune dysregulation, and personalized treatment approaches. A multidisciplinary approach involving immunologists, hematologists, infectious disease specialists, and geneticists is paramount to addressing the diagnostic and therapeutic challenges posed by this intriguing yet formidable clinical entity.