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

Donald C. Vinh | Zendy; Khalid Bin Dhuban | Zendy; Helen Mason | Zendy; Duncan Lejtenyi | Zendy; Sungmi Jung | Zendy; Donald C. Sheppard | Zendy; Damien Faury | Zendy; Nada Jabado | Zendy; Ciriaco A. Piccirillo | Zendy

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

Author(s) -

Donald C. Vinh,

Khalid Bin Dhuban,

Helen Mason,

Duncan Lejtenyi,

Sungmi Jung,

Donald C. Sheppard,

Damien Faury,

Nada Jabado,

Ciriaco A. Piccirillo

Publication year - 2011

Publication title -

clinical and vaccine immunology

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 1.649

H-Index - 77

eISSN - 1556-6811

pISSN - 1556-679X

DOI - 10.1128/cvi.05456-11

Subject(s) - hematopoietic stem cell transplantation , medicine , transplantation , hematopoietic cell , immunology , foxp3 , haematopoiesis , surgery , biology , stem cell , immune system , genetics

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 rapamycinin vitro .

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