Lymphoreticular tissue lesions in Steinbrinck-Chediak-Higashi syndrome.

Surgical and postmortem specimens of lymphoreticular tissue (organized and diffuse) from three patients with Steinbrinck-Chediak-Higashi syndrome were investigated by the light and electron microscope. Material before and after cancer chemotherapy was available. An extensive generalized hyperplasia of lymphoreticular tissues was observed in which several cell lines participated, including lymphocytes, histiocytes, and reticulum cells. Although this hyperplasia resembled neoplasia, histologic and cytologic details suggested it was only a reactive process. Therapy with prednisone and vincristine reduced the well differentiated cells in the lymphoreticular tissues, resulting in a more anaplastic population of immature lymphoid and reticulum cells. It is postulated that proliferation of the lymphoid and histiocytic stem cells is a reaction to the accumulation of materials normally degraded by phagocytes. This proliferation initially may be compensatory, but owing to persistent stimulation by nondegraded foreign materials it progresses to simulate neoplasia.