THE OCCURRENCE OF INTRACELLULAR IRON IN GAUCHER'S DISEASE

Abstract

In 5 cases, the cytoplasm of the characteristic cells stained diffusely with Prussian blue, indicating the presence of iron. This differed markedly from the numerous intra- and extracellular hemosiderin granules noted in a case of chronic hemolytic anemia. That the staining was not due to kerasin per se was inferred by the negative reaction of purified normal kerasin. The nongranular appearance of the cytoplasm together with a Prussian blue reaction of recrystallized ferritin even at a concentration of 0.3[mu]g/ ml suggested that the latter was the iron-containing compound demonstrated within the cells. Despite normal hemograms in 3, all 5 cases had evidence of minimal erythrophagocytosis which may have been responsible for at least some of the intracellular iron. It is also possible that the latter may reflect a specific disorder of iron metabolism resulting in profuse quantities of ferritin within the abnormal cells. Iron could not be demonstrated within reticulum cells from which the Gaucher cells are believed to develop. Evidently in the transformation from the former there is a significant accumulation of iron as well as kerasin.