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

Author:

Vinh Donald C.1,Bin Dhuban Khalid23,Mason Helen23,Lejtenyi Duncan4,Jung SungMi5,Sheppard Donald C.2,Faury Damien4,Jabado Nada4,Piccirillo Ciriaco A.123

Affiliation:

1. Division of Infectious Diseases, Division of Allergy and Clinical Immunology, Department of Medicine, and Department of Medical Microbiology, McGill University Health Centre, Montreal, QC, Canada

2. Department of Microbiology and Immunology, McGill University, Montreal, QC, Canada

3. FOCIS Center of Excellence, Research Institute of the McGill University Health Centre, Montreal, QC, Canada

4. Division of Pediatric Allergy and Clinical Immunology, Department of Pediatrics, McGill University Health Centre, Montreal, QC, Canada

5. Department of Pathology, McGill University Health Centre, Montreal, QC, Canada

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 .

Publisher

American Society for Microbiology

Subject

Microbiology (medical),Clinical Biochemistry,Immunology,Immunology and Allergy

Reference14 articles.

1. Rapamycin promotes expansion of functional CD4+ CD25+ FOXP3+ regulatory T cells of both healthy subjects and type 1 diabetic patients;Battaglia M;J. Immunol.,2006

2. Induction of autologous graft-versus-host disease: results of a randomized prospective clinical trial in patients with poor risk lymphoma;Bolaños-Meade J;Biol. Blood Marrow Transplant.,2007

3. Defect of regulatory T cells in patients with Omenn syndrome;Cassani B;J. Allergy Clin. Immunol.,2010

4. de VillartayJ-PSchwarzKVillaA. 2006. V(D)J recombination defects, p 153–168. In OchsHDSmithCIPuckJM (ed), Primary immunodeficiency diseases: a molecular and cellular approach. Oxford University Press, New York, NY.

5. Severe autologous GVHD after hematopoietic progenitor cell transplantation for multiple myeloma;Drobyski WR;Bone Marrow Transplant.,2009

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