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September 1941

THE GRANULES IN THE LEUKOCYTES IN GARGOYLISM

Author Affiliations

SAN FRANCISCO
From the Department of Pediatrics, University of California School of Medicine.

Am J Dis Child. 1941;62(3):489-491. doi:10.1001/archpedi.1941.02000150011002
Abstract

Gargoylism is a syndrome characterized by dwarfism, chondrodystrophic skeletal changes, infantilism, a facies resembling that of cretinism, thickening of the skin and the periarticular structures which limit joint extension, splenohepatomegaly and familial recurrence. Cloudy corneas often develop.

The white blood cells occasionally contain a hitherto undescribed type of granulation. This kind of cell has been encountered by my associates and me in 4 of 8 cases of gargoylism. The pathogenesis or significance of the granules (illustration) is unknown. They are found only in the white blood cells and occur much more often in the polymorphonuclears than in the lymphocytes or the monocytes. They are larger than the granules which are normally present in cells, are more numerous and usually are a dark lilac color when stained by the Giemsa-Wright technic. On other occasions, even in cells from the same patient, they may be much smaller than ordinary granules and may

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