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Cytoreductive conditioning for severe combined immunodeficiency--help or hindrance?

Abstract
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.
AuthorsAlexandra Laberko, Andrew R Gennery
JournalExpert review of clinical immunology (Expert Rev Clin Immunol) Vol. 11 Issue 7 Pg. 785-8 ( 2015) ISSN: 1744-8409 [Electronic] England
PMID26099343 (Publication Type: Editorial)
Topics
  • Allografts
  • Animals
  • B-Lymphocytes (immunology, pathology)
  • Hematopoietic Stem Cell Transplantation
  • Humans
  • Lymphocyte Depletion
  • Portraits as Topic
  • Severe Combined Immunodeficiency (immunology, pathology, therapy)
  • T-Lymphocytes (immunology, pathology)
  • Transplantation Conditioning (methods)

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