Abstract
An accelerated (lymphoma-like) phase is characteristic of Chédiak-Higashi syndrome (CHS), and it may develop in consequence of reactive rather than neoplastic response.
The accelerated phase was studied in two children with CHS by the electron microscopic and histological methods. The electron microscopy of the bone marrow in the accelerated phase and the histological examination at autopsy demonstrated prominent phagocytosis of erythrocytes and nucleated cells as well as mononuclear cell infiltration. The phagocytosis of autologous blood cells was particularly prominent in the tissues from the bone marrow, liver, spleen and lungs.
Peripheral neutrophils and monocytes of CHS were demonstrated to ingest autologous leukocytes and erythrocytes when they were mixed with bacteria in vitro. Such distorted phagocytic ability of CHS phagocytes appears to be related to the prominent phagocytosis of blood cells in the accelerated phase, being responsible for pancytopenia.
