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CD3+ T Cell Lineage Specific Maternal Engraftment in Pediatric Patient with Severe Combined Immunodeficiency Disorder

Received: 23 November 2021     Accepted: 15 December 2021     Published: 24 December 2021
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Abstract

Background: Transplacental Maternal Engraftment (TME) is common in patients with Severe Combined Immunodeficiency Disorder (SCID), however only a few are complicated by Graft Versus Host Disease (GVHD) prior to Hematopoietic Cell Transplantation (HCT). Objective: We will discuss a rare case of a SCID patient with complete TME at birth who later developed pre-HCT GVHD secondary to TME. Materials and Methods: Peripheral blood mononuclear cells or sorted cell populations are used for TME monitoring. Chimerism testing/engraftment analysis was performed by PCR based capillary electrophoresis to detect genetic polymorphisms in short tandem repeat loci. Results: SCID was diagnosed on newborn screen and the patient was prematurely born at 33 weeks of gestation. The patient had GVHD secondary to TME, which involved skin, liver, gut and bone marrow along with other clinical symptoms of SCID and treated with tacrolimus and methylprednisolone. The patient was transplanted three months after birth with an HLA identical sibling donor. Partial donor engraftment was seen in myeloid cells followed by B and T cell lineages from day +42 post transplantation. Testing sorted CD4+ and CD8+ T cells at day +42 revealed that the engrafted maternal CD3+ cells were exclusively of CD4+ phenotype, which represented 15% of circulating CD4+ T cells. Conclusion: Based on our findings, we suggest that CD3+ lineage specific T cells, presumably CD4+, might be the main contributor for pre-HCT GVHD secondary to TME.

Published in International Journal of Immunology (Volume 9, Issue 4)
DOI 10.11648/j.iji.20210904.12
Page(s) 73-78
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2021. Published by Science Publishing Group

Keywords

Chimerism, Maternal Engraftment, SCID, GVHD, CD3+ T Cell, HCT

References
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Cite This Article
  • APA Style

    Gansuvd Balgansuren, Nakita Shelton, Lois Regen, Dana McLendon, Susan Russell, et al. (2021). CD3+ T Cell Lineage Specific Maternal Engraftment in Pediatric Patient with Severe Combined Immunodeficiency Disorder. International Journal of Immunology, 9(4), 73-78. https://doi.org/10.11648/j.iji.20210904.12

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    ACS Style

    Gansuvd Balgansuren; Nakita Shelton; Lois Regen; Dana McLendon; Susan Russell, et al. CD3+ T Cell Lineage Specific Maternal Engraftment in Pediatric Patient with Severe Combined Immunodeficiency Disorder. Int. J. Immunol. 2021, 9(4), 73-78. doi: 10.11648/j.iji.20210904.12

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    AMA Style

    Gansuvd Balgansuren, Nakita Shelton, Lois Regen, Dana McLendon, Susan Russell, et al. CD3+ T Cell Lineage Specific Maternal Engraftment in Pediatric Patient with Severe Combined Immunodeficiency Disorder. Int J Immunol. 2021;9(4):73-78. doi: 10.11648/j.iji.20210904.12

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  • @article{10.11648/j.iji.20210904.12,
      author = {Gansuvd Balgansuren and Nakita Shelton and Lois Regen and Dana McLendon and Susan Russell and Paula Peterson and Ada Ng and Dylan Smith and Debra Cordell and Chris McFarland},
      title = {CD3+ T Cell Lineage Specific Maternal Engraftment in Pediatric Patient with Severe Combined Immunodeficiency Disorder},
      journal = {International Journal of Immunology},
      volume = {9},
      number = {4},
      pages = {73-78},
      doi = {10.11648/j.iji.20210904.12},
      url = {https://doi.org/10.11648/j.iji.20210904.12},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.iji.20210904.12},
      abstract = {Background: Transplacental Maternal Engraftment (TME) is common in patients with Severe Combined Immunodeficiency Disorder (SCID), however only a few are complicated by Graft Versus Host Disease (GVHD) prior to Hematopoietic Cell Transplantation (HCT). Objective: We will discuss a rare case of a SCID patient with complete TME at birth who later developed pre-HCT GVHD secondary to TME. Materials and Methods: Peripheral blood mononuclear cells or sorted cell populations are used for TME monitoring. Chimerism testing/engraftment analysis was performed by PCR based capillary electrophoresis to detect genetic polymorphisms in short tandem repeat loci. Results: SCID was diagnosed on newborn screen and the patient was prematurely born at 33 weeks of gestation. The patient had GVHD secondary to TME, which involved skin, liver, gut and bone marrow along with other clinical symptoms of SCID and treated with tacrolimus and methylprednisolone. The patient was transplanted three months after birth with an HLA identical sibling donor. Partial donor engraftment was seen in myeloid cells followed by B and T cell lineages from day +42 post transplantation. Testing sorted CD4+ and CD8+ T cells at day +42 revealed that the engrafted maternal CD3+ cells were exclusively of CD4+ phenotype, which represented 15% of circulating CD4+ T cells. Conclusion: Based on our findings, we suggest that CD3+ lineage specific T cells, presumably CD4+, might be the main contributor for pre-HCT GVHD secondary to TME.},
     year = {2021}
    }
    

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  • TY  - JOUR
    T1  - CD3+ T Cell Lineage Specific Maternal Engraftment in Pediatric Patient with Severe Combined Immunodeficiency Disorder
    AU  - Gansuvd Balgansuren
    AU  - Nakita Shelton
    AU  - Lois Regen
    AU  - Dana McLendon
    AU  - Susan Russell
    AU  - Paula Peterson
    AU  - Ada Ng
    AU  - Dylan Smith
    AU  - Debra Cordell
    AU  - Chris McFarland
    Y1  - 2021/12/24
    PY  - 2021
    N1  - https://doi.org/10.11648/j.iji.20210904.12
    DO  - 10.11648/j.iji.20210904.12
    T2  - International Journal of Immunology
    JF  - International Journal of Immunology
    JO  - International Journal of Immunology
    SP  - 73
    EP  - 78
    PB  - Science Publishing Group
    SN  - 2329-1753
    UR  - https://doi.org/10.11648/j.iji.20210904.12
    AB  - Background: Transplacental Maternal Engraftment (TME) is common in patients with Severe Combined Immunodeficiency Disorder (SCID), however only a few are complicated by Graft Versus Host Disease (GVHD) prior to Hematopoietic Cell Transplantation (HCT). Objective: We will discuss a rare case of a SCID patient with complete TME at birth who later developed pre-HCT GVHD secondary to TME. Materials and Methods: Peripheral blood mononuclear cells or sorted cell populations are used for TME monitoring. Chimerism testing/engraftment analysis was performed by PCR based capillary electrophoresis to detect genetic polymorphisms in short tandem repeat loci. Results: SCID was diagnosed on newborn screen and the patient was prematurely born at 33 weeks of gestation. The patient had GVHD secondary to TME, which involved skin, liver, gut and bone marrow along with other clinical symptoms of SCID and treated with tacrolimus and methylprednisolone. The patient was transplanted three months after birth with an HLA identical sibling donor. Partial donor engraftment was seen in myeloid cells followed by B and T cell lineages from day +42 post transplantation. Testing sorted CD4+ and CD8+ T cells at day +42 revealed that the engrafted maternal CD3+ cells were exclusively of CD4+ phenotype, which represented 15% of circulating CD4+ T cells. Conclusion: Based on our findings, we suggest that CD3+ lineage specific T cells, presumably CD4+, might be the main contributor for pre-HCT GVHD secondary to TME.
    VL  - 9
    IS  - 4
    ER  - 

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Author Information
  • Clinical Immunogenetics Laboratory, Seattle Cancer Care Alliance, Seattle, USA

  • Clinical Immunogenetics Laboratory, Seattle Cancer Care Alliance, Seattle, USA

  • Clinical Immunogenetics Laboratory, Seattle Cancer Care Alliance, Seattle, USA

  • Clinical Immunogenetics Laboratory, Seattle Cancer Care Alliance, Seattle, USA

  • Clinical Immunogenetics Laboratory, Seattle Cancer Care Alliance, Seattle, USA

  • Clinical Immunogenetics Laboratory, Seattle Cancer Care Alliance, Seattle, USA

  • Clinical Immunogenetics Laboratory, Seattle Cancer Care Alliance, Seattle, USA

  • Clinical Immunogenetics Laboratory, Seattle Cancer Care Alliance, Seattle, USA

  • Clinical Immunogenetics Laboratory, Seattle Cancer Care Alliance, Seattle, USA

  • Clinical Immunogenetics Laboratory, Seattle Cancer Care Alliance, Seattle, USA

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