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The image above shows myelinosomes, also known as “zebra bodies” for their appearance on tangential sectioning. Myelinosomes are seen in Fabry disease, a rare x-linked recessive genetic disorder caused by the defective activity of the enzyme alpha-galactosidase A. Renal, cardiac, and cerebrovascular involvement is common and can be fatal by the 4th decade without enzyme replacement therapy. Alpha galactosidase A deficiency results in accumulation of globotriaosylceramide within podocytes of glomeruli, tubules, myocytes, and endothelium. Globotriaosylceramide accumulation results in the formation of myelinosomes. Since myelinosomes are lipid-derived, they are maintained on osmium fixation, but are lost with exposure to xylenes in...