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Blood, Vol. 94 No. 11 (December 1), 1999:
pp. 3872-3882
Spontaneous Apoptosis in Lymphocytes From Patients With
Wiskott-Aldrich Syndrome: Correlation of Accelerated Cell Death and
Attenuated Bcl-2 Expression
Stephen L. Rawlings,
Gay M. Crooks,
David Bockstoce,
Lora W. Barsky,
Robertson Parkman, and
Kenneth I. Weinberg
From the Division of Research Immunology and Bone Marrow
Transplantation, Childrens Hospital Los Angeles, Los Angeles, CA.
Wiskott-Aldrich syndrome (WAS) is an X-linked recessive disorder
characterized by thrombocytopenia, eczema, and a progressive deterioration of immune function. WAS is caused by mutations in an
intracellular protein, WASP, that is involved in signal transduction and regulation of actin cytoskeleton rearrangement. Because immune dysfunction in WAS may be due to an accelerated destruction of lymphocytes, we examined the susceptibility to apoptosis of resting primary lymphocytes isolated from WAS patients in the absence of
exogenous apoptogenic stimulation. We found that unstimulated WAS
lymphocytes underwent spontaneous apoptosis at a greater frequency than
unstimulated normal lymphocytes. Coincident with increased apoptotic
susceptibility, WAS lymphocytes had markedly attenuated Bcl-2
expression, whereas Bax expression did not differ. A negative correlation between the frequency of spontaneous apoptosis and the
level of Bcl-2 expression was demonstrated. These data indicate that
accelerated lymphocyte destruction by spontaneous induction of
apoptosis may be one pathogenic mechanism by which the progressive immunodeficiency in WAS patients develops.
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