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.
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Authors | Alexandra Laberko, Andrew R Gennery |
Journal | Expert review of clinical immunology
(Expert Rev Clin Immunol)
Vol. 11
Issue 7
Pg. 785-8
( 2015)
ISSN: 1744-8409 [Electronic] England |
PMID | 26099343
(Publication Type: Editorial)
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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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