Glycosaminoglycan in the blood and renal tissue of a patient with nephroblastomatosis

Bilateral nephroblastomatosis was diagnosed in a 15-month-old white female. Prior to surgery, multiple peripheral blood smears (Wrights' stain) revealed an azurophilic staining extracellular material. When serum was added to a three percent acetic acid solution, a floccular, fibrous precipitate formed at the meniscus of the tube. Serum protein electrophoresis on cellulose acetate support media resulted in a distorted pattern which corrected to a normal pattern upon treatment with hyaluronidase. These peripheral blood abnormalities disappeared following a left nephrectomy. Quantitative chemical analysis of diseased renal tissue yielded 81 micrograms of readily extracted glycosaminoglycan (GAG) per gram of tissue. The importance of abnormal glycosaminoglycan production in patients with malignant disease is discussed both in terms of clinical importance and possible roles of cell exudates.