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Hematopoietic Stem Cell Transplantation in Late-onset X-linked Chronic Granulomatous Disease in a Female Carrier.

Authors :
Trevisan, Matteo
Kang, Elizabeth M.
CGD Consortium
Salton, Francesco
Ruaro, Barbara
Torregiani, Chiara
Confalonieri, Paola
Naviglio, Samuele
Valencic, Erica
Gabrielli, Marco
Parta, Mark
Kelly, Corin
Notarangelo, Luigi D.
Malech, Harry L.
Tommasini, Alberto
Confalonieri, Marco
Source :
Journal of Clinical Immunology; Oct2022, Vol. 42 Issue 7, p1396-1399, 4p
Publication Year :
2022

Abstract

HSCT remains the main definitive treatment for X-linked CGD, yet older patients are at greater risk of transplant-related complications and mortality [[3]]. Recombinant interferon gamma augments phagocyte superoxide production and X-chronic granulomatous disease gene expression in X-linked variant chronic granulomatous disease. This suggests that matched HSCT with non-myeloablative conditioning with low dose busulfan and alemtuzumab, combined with an increased stem cell dose and post-transplant cyclophosphamide, may represent a curative option even in high-risk CGD patients at an advanced age. [Extracted from the article]

Details

Language :
English
ISSN :
02719142
Volume :
42
Issue :
7
Database :
Complementary Index
Journal :
Journal of Clinical Immunology
Publication Type :
Academic Journal
Accession number :
160293844
Full Text :
https://doi.org/10.1007/s10875-022-01310-9