MORPHOLOGICAL ABNORMALITIES IN THE LYMPHOCYTES OF PATIENTS WITH THE WISKOTT-ALDRICH SYNDROME

  • 1 December 1986
    • journal article
    • research article
    • Vol. 68  (6) , 1329-1332
Abstract
Lymphocytes from 18 patients with the Wiskott-Aldrich Syndrome (WAS) were examined by scanning electron microscopy (SEM). Most peripheral blood lymphocytes from normal individuals are covered with slender microvillus projections, but a large proportion of lymphocytes from WAS patients were found to be relatively devoid of microvilli. A lymphocyte morphology scoring system was developed to quantify the density of microvilli: Grade 4 classified those lymphocytes with > 75% of the surface covered with microvilli with progressive decrements to grade 1, which were those without microvilli. The mean lymphocyte morphology score of eight normal individuals was 3.62 .+-. .22. The mean lymphocyte score of WAS patients was substantially lower (2.89 .+-. .27, P < .001). In addition, WAS lymphocytes often were qualitatively abnormal, with short, blunted microvilli. These morphological criteria were used to diagnose WAS from the cord blood lymphocytes of one ''''at-risk'''' patient. Thus, WAS is the first primary immunodeficiency in which morphological abnormalities have been identified that can aid in diagnosis.