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The Wiskott-Aldrich Syndrome
Immunopathologic Mechanisms and a Long-Term Survival
- M. A. J. MANDL, M.B.;
- J. I. WATSON, M.D., F.R.C.P.(C); and
- B. ROSE, M.D., PH.D., F.A.C.P.
- Requests for reprints should be addressed to M. A. J. Mandl, M.B., Department of Immunochemistry and Allergy, Royal Victoria Hospital, Montreal 2, Quebec, Canada.
SUMMARY
A case is reported of a 24-year-old man who presented with clinical and laboratory findings compatible with the Wiskott-Aldrich syndrome. The additional features associated with this entity since its original description are reviewed and the necessity for enlarging the concepts of the syndrome discussed. It is felt that the present case further enlarges the spectrum of disease seen in these patients. In addition, further evidence of the nature of at least one involved immunologic deficit is presented. It appears that the Wiskott-Aldrich syndrome represents a situation in which cellular mechanisms are impaired in varying degree while the immunoglobulin system is intact or only slightly abnormal.
Article and Author Information
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From the Harry Webster Thorp Laboratories for the Division of Immunochemistry and Allergy, Royal Victoria Hospital, McGill University, Montreal, Quebec, Canada.
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Dr. Mandl was supported in this study by clinical fellowship 5T1-A1-71-97, U. S. Public Health Service, Washington, D. C.
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- Received November 27, 1967.
- Accepted January 25, 1968.
