Abstract
Historically, severe combined immune deficiency(SCID) has been a model disease for the development of innovative transplantation procedures, e.g., the first successful histocompatible bone marrow transplants (BMT), haploidentical T-cell depleted BMT, or matched unrelated donor BMT [1]. Because patients with SCID have defective immunity, their ability to reject transplanted marrow is limited. This characteristic has made it possible to achieve engraftment from a variety of sources. In histocompatible BMT, pre-transplant cytotoxic conditioning is not required to ensure engraftment, although conditioning is usually needed for histoincompatible BMT [2]. Transplants without conditioning result is a state of chimerism with donor T lymphocytes, donor or recipient B lymphocytes, and recipient haematopoietic cells being found after BMT. The engraftment of only recipient T lymphocytes in patients with SCID, who did not receive pre-transplant conditioning, establishes that normal donor T lymphocyte progenitors have a proliferative advantage over the recipient SCID T lymphocyte progenitors.
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© 1995 Springer Science+Business Media Dordrecht
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Weinberg, K.I. (1995). Gene Therapy for Primary Immune Deficiencies. In: Sibinga, C.T.S., Das, P.C., Briët, E. (eds) Hereditary Diseases and Blood Transfusion. Developments in Hematology and Immunology, vol 30. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-2017-7_14
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DOI: https://doi.org/10.1007/978-1-4615-2017-7_14
Publisher Name: Springer, Boston, MA
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