A case of generalized amyloidosis associated with cyclic neutropenia
T Shiomura, Y Ishida, N Matsumoto, K Sasaki, T Ishihara and S Miwa
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.
Volume 54,
Issue 3,
pp. 628-635,
09/01/1979
Copyright © 1979 by The American Society of Hematology
