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Lookup NU author(s): Professor Andrew GenneryORCiD
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Use of chemotherapy-based conditioning-facilitated engraftment in patients with severe combined immunodeficiency (SCID) is contentious. In T- and NK lymphocyte-negative, B-lymphocyte-positive (T-B+NK+) and T-B-NK+ SCID, the osteo-medullary space is occupied by recipient hematopoietic stem cells and mature B-lymphocytes. The thymic niche is empty in T-B+NK+ SCID but fully occupied by developmentally arrested T- lymphocyte precursors in T-B-NK+ SCID. The outcome of infusion of donor stem cells differs and is dependent on genetic defect and the lymphocyte developmental arrest stage. At best, donor hematopoietic stem cell osteo-medullary engraftment induces normal B-lymphocyte function and long-term thymopoiesis; at worst, peripheral expansion of donor T- lymphocytes from the stem cell source results in a restricted T- lymphocyte receptor repertoire with possible B-lymphocyte failure. Conditioning improves immunoreconstitution but causes short- and long-term toxicities, and increased mortality. Newborn screening for SCID will propel the search for safe, effective methods of achieving donor cell engraftment and full immunoreconstitution without toxic sequalae.
Author(s): Laberko A, Gennery AR
Publication type: Editorial
Publication status: Published
Journal: Expert Review of Clinical Immunology
Print publication date: 01/07/2015
Online publication date: 22/06/2015
Acceptance date: 01/01/1900
ISSN (print): 1744-666X
ISSN (electronic): 1744-8409
Publisher: Taylor & Francis