Hematopoietic Cell Transplantation in Patients With Primary Immune Regulatory Disorders (PIRD): A Primary Immune Deficiency Treatment Consortium (PIDTC) Survey.

Frontiers in Immunology
Alice Y ChanTroy Torgerson

Abstract

Primary Immune Regulatory Disorders (PIRD) are an expanding group of diseases caused by gene defects in several different immune pathways, such as regulatory T cell function. Patients with PIRD develop clinical manifestations associated with diminished and exaggerated immune responses. Management of these patients is complicated; oftentimes immunosuppressive therapies are insufficient, and patients may require hematopoietic cell transplant (HCT) for treatment. Analysis of HCT data in PIRD patients have previously focused on a single gene defect. This study surveyed transplanted patients with a phenotypic clinical picture consistent with PIRD treated in 33 Primary Immune Deficiency Treatment Consortium centers and European centers. Our data showed that PIRD patients often had immunodeficient and autoimmune features affecting multiple organ systems. Transplantation resulted in resolution of disease manifestations in more than half of the patients with an overall 5-years survival of 67%. This study, the first to encompass disorders across the PIRD spectrum, highlights the need for further research in PIRD management.

References

Sep 17, 2013·The Journal of Allergy and Clinical Immunology·Peter D ArkwrightRobert F Wynn
Jan 18, 2015·The Journal of Allergy and Clinical Immunology·Claudia WehrUNKNOWN Inborn Errors Working Party of the European Society for Blood and Marrow Transplantation and the European Society for Immuno
Apr 23, 2016·The Journal of Allergy and Clinical Immunology·Mary A SlatterTroy R Torgerson
Jun 6, 2016·The Journal of Allergy and Clinical Immunology·Linda M GriffithUNKNOWN workshop participants
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May 16, 2017·The Journal of Allergy and Clinical Immunology·Markus G SeidelUNKNOWN Inborn Errors Working Party of the European Group for Blood and Marrow Transplantation
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Dec 12, 2017·Journal of Clinical Immunology·Aziz BousfihaKathleen E Sullivan
Dec 16, 2017·The Journal of Allergy and Clinical Immunology·Federica BarzaghiUNKNOWN Primary Immune Deficiency Treatment Consortium (PIDTC) and the Inborn Errors Working Party (IEWP) of the European Society fo
May 8, 2018·The Journal of Allergy and Clinical Immunology·Charlotte SchwabBodo Grimbacher
Dec 5, 2019·Frontiers in Pediatrics·Shahrzad BakhtiarEleonora Gambineri

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Citations

Sep 18, 2020·Current Opinion in Allergy and Clinical Immunology·Alice Y Chan, Troy R Torgerson
Nov 11, 2020·Transplant Infectious Disease : an Official Journal of the Transplantation Society·Federica ForlaniniMorna J Dorsey
Dec 18, 2020·Immunology Letters·Suran L FernandoJamma Li
Feb 4, 2021·Annual Review of Immunology·Erica G Schmitt, Megan A Cooper
Jul 13, 2021·Frontiers in Pediatrics·Romane ThouenonSven Kracker
Jul 15, 2021·Current Opinion in Rheumatology·Mario Abinun, Mary A Slatter
Jul 7, 2021·Bone Marrow Transplantation·A C LankesterUNKNOWN Inborn Errors Working Party of the European Society for Blood and Marrow Transplantation and the European Society for Immune

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Software Mentioned

Statistical Analysis Software ( SAS )

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