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Hematopoietic Stem Cell Transplantation Resolves the Immune Deficit Associated with STAT3-Dominant-Negative Hyper-IgE Syndrome by Stephanie C. Harrison; Christo Tsilifis; Mary A. Slatter; Zohreh Nademi; Austen Worth; Paul Veys; Mark J. Ponsford; Stephen Jolles; Waleed Al-Herz; Terence Flood; Andrew J. Cant; Rainer Doffinger; Gabriela Barcenas-Morales; Ben Carpenter; Rachael Hough; Ásgeir Haraldsson; Jennifer Heimall; Bodo Grimbacher; Mario Abinun; Andrew R. Gennery is a Immunology and Microbiology article available to read on EtoBox.

What is Hematopoietic Stem Cell Transplantation Resolves the Immune Deficit Associated with STAT3-Dominant-Negative Hyper-IgE Syndrome about?

Autosomal dominant hyper-IgE syndrome caused by dominant-negative loss-of-function mutations in signal transducer and activator of transcription factor 3 (__STAT3__) (STAT3-HIES) is a rare primary immunodeficiency with multisystem pathology. The quality of life in patients with STAT3-HIES is determined by not only the progressive, life-limiting pulmonary disease, but also significant skin disease including recurrent infections and abscesses requiring surgery. Our early report indicated that hematopoietic stem cell transplantation might not be effective in patients with STAT3-HIES, although a few subsequent reports have reported successful outcomes. We update on progress of our patient now with over 18 years of follow-up and report on an additional seven cases, all of whom have survived despite demonstrating significant disease-related pathology prior to transplant. We conclude that effective cure of the immunological aspects of the disease and stabilization of even severe lung involvement may be achieved by allogeneic hematopoietic stem cell transplantation. Recurrent skin infections and abscesses may be abolished. Donor T~H~17 cells may produce comparable levels of IL17A to health

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Author

Stephanie C. Harrison; Christo Tsilifis; Mary A. Slatter; Zohreh Nademi; Austen Worth; Paul Veys; Mark J. Ponsford; Stephen Jolles; Waleed Al-Herz; Terence Flood; Andrew J. Cant; Rainer Doffinger; Gabriela Barcenas-Morales; Ben Carpenter; Rachael Hough; Ásgeir Haraldsson; Jennifer Heimall; Bodo Grimbacher; Mario Abinun; Andrew R. Gennery

Publisher

Springer Science and Business Media LLC

Published

2021

Language

EN

Field

Immunology and Microbiology (Life Sciences)