Wiskott-Aldrich syndrome: histopathologic findings in the lymph nodes and spleens of 15 patients.

Seventeen lymph nodes and 13 spleens from 15 patients with the Wiskott-Aldrich syndrome were examined histologically. The material included both biopsy and autopsy specimens. Consistent findings included depletion of small lymphocytes from T cell areas (all cases), prominence of the reticulum cell stroma (all cases), the presence of atypical plasma cells with and without plasmacytosis (16 lymph nodes and 11 spleens), and extramedullary hematopoiesis (13 lymph nodes and 9 spleens). Less frequent features noted were tissue eosinophilia, hemophagocytosis, focal fibrosis, and progressive depletion of germinal centers. One case with a unique abundance of transformed lymphocytes is described.