Abstract
We have previously reported abnormalities in the membrane glycoproteins of the T lymphocytes and platelets of WAS patients. Deficiencies in a 115,000 dalton lymphocyte glycoprotein (GPL-115) appear to be diagnostic of WAS. To determine if the membrane glycoprotein abnormalities have morphological consequences, normal and WAS peripheral blood lymphocytes (PBL) and thymocytes were fixed in 1.2% glutaraldehyde and examined by scanning electron microscopy (SEM). Using a 1 to 4 grading scale based upon the character of the lymphocyte surface projections (4 = villus projections on > 75% of lymphocyte surface area; 3 = villus projections on < 75% of surface area; 2 = ridge projections; 1 = no projections), normal PEL had an average score of 3.60±.10(SE); thymocytes, 2.00±.02; WAS PBL, 2.76±.07 (n = 14). The decreased score of WAS lymphocytes was due to a decrease in the percentage of cells with villus projections and an increase in the percentage of cells with ridge or no projections. SEM has been used to confirm the diagnosis of WAS on the cord blood lymphocytes of one patient. WAS represents the first lymphoid immunodeficiency in which morphological abnormalities have been identified that can be used for diagnostic purposes.
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Cairns, L., Kenney, D., Neustein, H. et al. 961 THE MORPHOLOGICAL DIAGNOSIS OF THE WISKOTT-ALDRICH SYNDROME (WAS). Pediatr Res 19, 271 (1985). https://doi.org/10.1203/00006450-198504000-00991
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DOI: https://doi.org/10.1203/00006450-198504000-00991