THE LYMPH-NODE PATHOLOGY OF OMENNS SYNDROME

Omenn's syndrome is a rare, usually fatal immunologic disorder of infa ncy characterized by recurrent infections, skin lesion, lymphadenopath y, peripheral blood lymphocytosis, and eosinophilia. Histologic evalua tion of a lymph node revealed total effacement of the microscopic arch itecture resulting from a diffuse proliferation of interdigitating ret iculum cells and a depletion of B lymphocytes. The lymph node lacked a distinct cortex and had no follicle formation. The most striking feat ure was a diffuse hyperplasia of S-100-protein-positive nonphagocytic reticulum cells with large, pale Langerhans-like nuclei. Ultrastructur al examination identified these cells to be interdigitating reticulum cells. The lymphocytes were small and predominantly of the CD8 cytotox ic/suppressor cell type.