Acquired Omenn-like syndrome, a novel posttransplant autoaggression syndrome reversed by rapamycin.

[omenn syndrome]

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 FOXP 3 (+) regulatory T cell defect that coincided with hyperinflammatory T cell responses which were reversible with rapamycin in vitro .