Case report: EBV-related eye orbits and sinuses lymphohistiocytic infiltration responsive to rituximab in a patient with X lymphoproliferative syndrome type 1

Case report: EBV-related eye orbits and sinuses lymphohistiocytic infiltration responsive to rituximab in a patient with X lymphoproliferative syndrome type 1

Background and aimsX lymphoproliferative syndrome type 1 (XLP1) is a rare inborn error of immunity due to mutations of SH2D1A, encoding for slam-associated protein (SAP). The clinical phenotype includes severe mononucleosis, hemophagocytic lymphohistiocytosis (HLH), and B-cell lymphomas.MethodsWe re...

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Main Authors: Giuliana Giardino, Vittoria Lanni, Massimo Mascolo, Daniela Russo, Emilia Cirillo, Roberta Romano, Francesca Cillo, Laura Grilli, Maria Rosaria Prencipe, Adriana Iuliano, Giovanni Uccello, Carmela De Fusco, Giuseppe Menna, Giulia Scalia, Giuseppe Portella, Claudio Pignata
Format: Article
Language:English
Published: Frontiers Media S.A. 2024-04-01
Series:Frontiers in Immunology
Subjects:
X lymphoproliferative syndrome type 1
eye lymphohistiocytic infiltration
EBV
rituximab
SH2D1A
Online Access:https://www.frontiersin.org/articles/10.3389/fimmu.2024.1370991/full

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https://www.frontiersin.org/articles/10.3389/fimmu.2024.1370991/full

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