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American Journal of Pathology, Vol 103, 247-262, Copyright © 1981 by American Society for Investigative Pathology

REGULAR ARTICLES

Light- and electron-microscopic histochemistry of Fabry's disease

T Faraggiana, J Churg, E Grishman, L Strauss, A Prado, DF Bishop, E Schuchman and RJ Desnick

A histochemical study was performed on light- and electron-microscopic level in a case of Fabry's disease. The patient underwent kidney transplantation for renal failure and died of heart failure 6 months later. Patient's tissues were studied at the light- and electron- microscopic levels with various embedding and staining techniques for lipids and carbohydrates. Two peroxidase-labeled lectins (from Ricinus communis and from Bandeiraea simplicifolia) known to have affinity for alpha- and beta-D-galactose, were strongly reactive with the storage material on frozen sections. The ultrahistochemical and extraction tests showed that the typical granules had a variable reactivity and morphologic characteristics in different cells, probably reflecting different composition. A small number of typical deposits were also observed in the transplanted kidney. This is the first reported case of recurrence of the storage disease in the allograft. Of interest was also the fact that the patient's blood inhibited normal alpha- galactosidase activity, suggesting a possible inhibitor-related mechanism in the pathogenesis of the recurrence.


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Copyright © 1981 by the American Society for Investigative Pathology.