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Morphological abnormalities in the lymphocytes of patients with the
Wiskott-Aldrich syndrome
D Kenney, L Cairns, E Remold-O'Donnell, J Peterson, FS Rosen and R Parkman
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 greater than 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 less than .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.
Volume 68,
Issue 6,
pp. 1329-1332,
12/01/1986
Copyright © 1986 by The American Society of Hematology
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