WHIPPLE'S DISEASE

Summary The clinical course and autopsy findings in three cases of Whipple's disease are described. The basic abnormality is considered to be a proliferation of mucoprotein‐producing reticulo‐endothelial cells, maximally concentrated in the small intestinal lymphatic system, but occurring at other sites including peripheral lymph nodes where, in the earliest stages, these cells can be seen arising in the germ centres. Transitions demonstrable between RNA‐containing cells and cells containing mucoprotein are considered to indicate synthesis of the mucoprotein, rather than its phagocytosis by the proliferating reticulo‐endothelial cells. Collagenization of lymph nodes and lymphatics is believed to be a result of the activity of these cells and responsible for the observed obstructive phenomena. The stimulus responsible for the proliferation of the mucoprotein‐synthesizing cells is unknown. It may represent a highly differentiated neoplastic process. Again, it may be the outcome of some cellular defect—e.g., enzymatic—whereby there is an abnormal accumulation of a normally synthesized mucoprotein. Response to steroid therapy in this condition is compatible with the known reticulo‐endothelial suppressive action of adrenal cortical steroids. However, this response is not sustained.