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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.
Ann Intern Med. 1968;68(5):1050-1059. doi:10.7326/0003-4819-68-5-1050
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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.

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