Insights into absence of lymphoma despite fulminant Epstein-Barr virus infection in patients with XIAP deficiency.

ABSTRACT: X-linked Lymphoproliferative Syndromes (XLP), arising from mutations in SH2D1A or XIAP genes, are characterized by fulminant Epstein-Barr virus (EBV) infection. Lymphomas occur frequently in XLP-1 and in other congenital conditions with heightened EBV susceptibility, but not in XLP-2. Why XLP-2 patients are apparently protected from EBV-driven lymphomagenesis remains a key open question. To gain insights, newly EBV-infected versus receptor-stimulated primary B cells from XLP-2 patients or with XIAP CRISPR editing were compared with healthy controls. XIAP perturbation impeded outgrowth of newly EBV-infected B cells, but not of CD40 ligand and interleukin-21-stimulated B cells. XLP-2-deficient B cells showed significantly lower EBV transformation efficiency than cells from healthy controls.

SUBMITTER: Sun Y 

PROVIDER: S-EPMC12406724 | biostudies-literature | 2025 Aug

REPOSITORIES: biostudies-literature