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American Society for Microbiology, Clinical and Vaccine Immunology, 1(19), p. 109-112, 2012

DOI: 10.1128/cvi.05456-11

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Acquired Omenn-Like Syndrome, a Novel Posttransplant Autoaggression Syndrome Reversed by Rapamycin

Journal article published in 2011 by Donald C. Vinh ORCID, Khalid Bin Dhuban, Helen Mason, Duncan Lejtenyi, SungMi Jung, Donald C. Sheppard, Damien Faury, Nada Jabado, Ciriaco A. Piccirillo
This paper is made freely available by the publisher.
This paper is made freely available by the publisher.

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Abstract

ABSTRACT Graft-versus-host disease is uncommon in autologous hematopoietic cell transplantation (HCT) and is typically brief and mild. We report unusual, protracted, and severe Omenn syndrome-like autoaggression following autologous HCT. We identified a profound FOXP3 + regulatory T cell defect that coincided with hyperinflammatory T cell responses which were reversible with rapamycin in vitro .