Chronic mucocutaneous candidiasis with macrophage dysfunction, a plasma inhibitor, and co-existent aplastic anemia

A 56-year-old man developed chronic mucocutaneous candidiasis (MCC) and pernicious anemia. Nine years later he developed aplastic anemia which ultimately was fatal. A small thymoma was found at autopsy. He was anergic and his mononuclear leukocytes (MNL) failed to undergo a proliferative response in culture to soluble antigens. His monocytes did not mediate a proliferative response by lymphocytes from sensitized control donors when stimulated with Monilia albicans antigen but did mediate a mixed leukocyte reaction normally. His plasma contained a poten inhibitor of -3H-thymidine incorporation by sensitized control MNL when stimulated with soluble antigens but was not inhibitory of the mixed leukocyte reaction, lymphoproliferative responses to plant mitogens, and was not shown either in vivo or in vitro to depress hematopoiesis. Patient lymphocytes were responsive to plant mitogens, Monilia antigen, in the mixed leukocyte reaction, and produced macrophage migration inhibitory factor in response to Monilia antigen. After plasmapheresis, delayed hypersensitivity and lymphoproliferative responses to soluble antigens were temporarily restored. This case implicates the macrophage in the pathogenesis of MCC and demonstrates some consequences of chronic monocyte dysfunction. The inhibitor of some expressions of cell-mediated immunity was removed by plasmapheresis.