Published in:
Open Access
01-04-2024 | CAR T-Cell Therapy | Letter to Editor
Monozygotic Twins with MAGT1 Deficiency and Epstein–Barr virus-positive Classic Hodgkin Lymphoma Receiving anti-CD30 CAR T-cell Immunotherapy: A case Report
Authors:
Jiachen Wang, Mi Zhou, Jianfeng Zhou, Min Xiao, Liang Huang
Published in:
Journal of Clinical Immunology
|
Issue 4/2024
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Excerpt
Epstein-Barr virus (EBV)-positive classical Hodgkin lymphoma (cHL) is the most common malignancy in patients with “X-linked immunodeficiency with magnesium defect, EBV infection, and neoplasia” (XMEN). Aberrant expression of MAGT1 leads to XMEN, a congenital disorder of combined primary immunodeficiency (PID) characterized by increased susceptibility to chronic EBV infection and EBV-associated lymphoproliferation [
1]. Patients with variants in
MAGT1 suffer from an N-linked glycosylation defect, resulting in low CD4
+ cell counts with an inverted CD4:CD8 ratio, reduced expression of NKG2D (a natural killer (NK)-cell activating receptor) on NK cells and cytotoxic T lymphocytes (CTLs), and impaired T-cell activation through NKG2D [
2]. …