A case of generalized amyloidosis associated with cyclic neutropenia

A case of generalized amyloidosis associated with cyclic neutropenia is presented. A 24-yr-old female with cyclic neutropenia died from intestinal obstruction caused by necrosis and perforation of the small intestine. Post-mortem examination revealed generalized amyloidosis involving almost all organs. Amyloid deposits were prominent, especially in the alimentary tract, kidneys, spleen, and small blood vessels. As has been suggested in gray collie dogs with congenital cyclic neutropenia known to develop secondary amyloidosis in adulthood, an increase of antigenic stimulation during the intermittent bouts of acute infections may be one of the factors responsible for the development of secondary amyloidosis in this case. Although the association of amyloidosis and cyclic neutropenia in man has rarely been described, it is probable that amyloidosis is not a rare complication of human cyclic neutropenia, considering that patients with this hematologic disorder are chronically exposed to excessive antigenic stimulation.