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Long-term outcome of non-ablative booster BMT in patients with SCID

Publication ,  Journal Article
Teigland, CL; Parrott, RE; Buckley, RH
Published in: Bone Marrow Transplantation
2013

SCID is a fatal syndrome caused by mutations in at least 13 different genes. It is characterized by the absence of T cells. Immune reconstitution can be achieved through nonablative related donor BMT. However, the first transplant may not provide sufficient immunity. In these cases, booster transplants may be helpful. A prospective/retrospective study was conducted of 49 SCID patients (28.7% of 171 SCIDs transplanted over 30 years) who had received booster transplants to define the long-term outcome, factors contributing to a need for a booster and factors that predicted success. Of the 49 patients, 31 (63%) are alive for up to 28 years. Age at initial transplantation was found to have a significant effect on outcome (mean of 194 days old for patients currently alive, versus a mean of 273 days old for those now deceased, P=0.0401). Persistent viral infection was present in most deceased booster patients. In several patients, the use of two parents as sequential donors resulted in striking T-and B-cell immune reconstitution. A majority of the patients alive today have normal or adequate T-cell function and are healthy. Nonablative booster BMT can be lifesaving for SCID. © 2013 Macmillan Publishers Limited. All rights reserved.

Duke Scholars

Published In

Bone Marrow Transplantation

DOI

ISSN

0268-3369

Publication Date

2013

Volume

48

Issue

8

Start / End Page

1050 / 1055

Related Subject Headings

  • Immunology
  • 1112 Oncology and Carcinogenesis
  • 1103 Clinical Sciences
 

Citation

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Teigland, C. L., Parrott, R. E., & Buckley, R. H. (2013). Long-term outcome of non-ablative booster BMT in patients with SCID. Bone Marrow Transplantation, 48(8), 1050–1055. https://doi.org/10.1038/bmt.2013.6
Teigland, C. L., R. E. Parrott, and R. H. Buckley. “Long-term outcome of non-ablative booster BMT in patients with SCID.” Bone Marrow Transplantation 48, no. 8 (2013): 1050–55. https://doi.org/10.1038/bmt.2013.6.
Teigland CL, Parrott RE, Buckley RH. Long-term outcome of non-ablative booster BMT in patients with SCID. Bone Marrow Transplantation. 2013;48(8):1050–5.
Teigland, C. L., et al. “Long-term outcome of non-ablative booster BMT in patients with SCID.” Bone Marrow Transplantation, vol. 48, no. 8, 2013, pp. 1050–55. Scival, doi:10.1038/bmt.2013.6.
Teigland CL, Parrott RE, Buckley RH. Long-term outcome of non-ablative booster BMT in patients with SCID. Bone Marrow Transplantation. 2013;48(8):1050–1055.

Published In

Bone Marrow Transplantation

DOI

ISSN

0268-3369

Publication Date

2013

Volume

48

Issue

8

Start / End Page

1050 / 1055

Related Subject Headings

  • Immunology
  • 1112 Oncology and Carcinogenesis
  • 1103 Clinical Sciences