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

Gansuvd Balgansuren; Nakita Shelton; Lois Regen; Dana McLendon; Susan Russell; Paula Peterson; Ada Ng; Dylan Smith; Debra Cordell; Chris McFarland

doi:doi:10.11648/j.iji.20210904.12

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

Gansuvd Balgansuren, Nakita Shelton, Lois Regen, Dana McLendon, Susan Russell, Paula Peterson, Ada Ng, Dylan Smith, Debra Cordell, Chris McFarland

Published in International Journal of Immunology (Volume 9, Issue 4)

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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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

Gansuvd Balgansuren

Clinical Immunogenetics Laboratory, Seattle Cancer Care Alliance, Seattle, USA
Nakita Shelton

Clinical Immunogenetics Laboratory, Seattle Cancer Care Alliance, Seattle, USA
Lois Regen

Clinical Immunogenetics Laboratory, Seattle Cancer Care Alliance, Seattle, USA
Dana McLendon

Clinical Immunogenetics Laboratory, Seattle Cancer Care Alliance, Seattle, USA
Susan Russell

Clinical Immunogenetics Laboratory, Seattle Cancer Care Alliance, Seattle, USA
Paula Peterson

Clinical Immunogenetics Laboratory, Seattle Cancer Care Alliance, Seattle, USA
Ada Ng

Clinical Immunogenetics Laboratory, Seattle Cancer Care Alliance, Seattle, USA
Dylan Smith

Clinical Immunogenetics Laboratory, Seattle Cancer Care Alliance, Seattle, USA
Debra Cordell

Clinical Immunogenetics Laboratory, Seattle Cancer Care Alliance, Seattle, USA
Chris McFarland

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

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Plain Text BibTeX RIS

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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