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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 .

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