Wiskott-Aldrich Syndrome With 18-Year Survival

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Abstract
An 18-year-old boy with Wiskott-Aldrich syndrome has severe symptoms of thrombocytopenia, recurrent infections, and atopic eczema. We believe he is the fifth oldest patient described with Wiskott-Aldrich syndrome. Recently, a malignant lymphoma of the histiocytic type appeared in the skin, while he was receiving transfer factor. To our knowledge, he is the only reported patient with lymphoma in the skin, but four other patients with Wiskott-Aldrich syndrome have developed malignant lymphoreticular lymphoma during transfer factor therapy. Detailed immunologic studies show failure to make a sustained antibody response to various antigens, lack of delayed hypersensitivity responsiveness, and failure of proliferative response to antigens in in vitro cultures. The IgE and IgA levels were high, and the IgM and IgG levels were low. Although clinical improvement followed transfer factor therapy, development of the malignant lymphoma was not prevented.