Persistent vacuoles were seen in the protoplasm of granulocytes, monocytes and occasional lymphocytes of 2 sisters suffering from ichthyosis. In the bone marrow these vacuoles were found in the cytoplasm of promyelocytes, myelocytes, metamyelocytes and rarely in plasma cells. They were not observed in blasts or in red cells and thrombocytes and their precursors.
With the help of cytochemical staining technics and fluorescence microscopy studies, it was determined that the vacuoles contained lipids. A similar abnormality of leukocytes was described previously by Jordans in 2 brothers suffering from muscular dystrophy.
Ichthyosis was transmitted in this family in an autosomal recessive way.
- Submitted September 14, 1965.
- Accepted December 10, 1965.
- © 1966 by American Society of Hematology, Inc.