When treating patients with EBV encephalitis, the possibility of XLP should be considered. Once the diagnosis of XLP is made, aggressive treatment such as rituximab, and other immunosuppressive agents are desired for rapid transition to HSCT.
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http://dx.doi.org/10.1002/ccr3.7949 | DOI Listing |
BMC Infect Dis
December 2024
National Center for Respiratory Medicine, China-Japan Friendship Hospital, Beijing, 100029, China.
X-linked lymphoproliferative disease (XLP) is a rare primary immunodeficiency with susceptibility and vulnerability to Epstein-Barr virus (EBV) infection. Most patients were diagnosed in early childhood and do not survive into adulthood. Here we reported an adult-onset XLP patient presenting with fever, dyspnea, and pulmonary nodules, mimicking respiratory infection at disease onset.
View Article and Find Full Text PDFPediatr Blood Cancer
February 2025
Pediatric Hematology and Oncology, Mayo Clinic, Rochester, Minnesota, USA.
Cureus
August 2024
Pediatrics, Dr. D. Y. Patil Medical College, Hospital & Research Centre, Dr. D. Y. Patil Vidyapeeth (Deemed to be University), Pune, IND.
Mol Ther Methods Clin Dev
December 2024
Department of Molecular & Medical Pharmacology, University of California, Los Angeles, Los Angeles, CA 90095, USA.
X-linked lymphoproliferative disease (XLP1) results from gene mutations affecting the SLAM-associated protein (SAP). A regulated lentiviral vector (LV), XLP-SMART LV, designed to express SAP at therapeutic levels in T, NK, and NKT cells, is crucial for effective gene therapy. We experimentally identified 34 genomic regulatory elements of the gene and designed XLP-SMART LVs to emulate the lineage and stage-specific control of SAP.
View Article and Find Full Text PDFMediterr J Hematol Infect Dis
May 2024
Department of Hematology and Oncology, Beijing Jingdu Children's Hospital, China.
The aim of this study was to investigate the prognostic factors of haploid hematopoietic stem cell transplantation in the treatment of X-linked lymphoproliferative syndrome. Seven children with X-linked lymphoproliferative syndrome diagnosed by XIAP gene analysis were enrolled. The conditioning regimens were tolerated in all seven patients, and the median time of neutrophil engraftment was 10 days (8-13 days), and that of platelet engraftment was 21 days (14-24 days).
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