Long-term survival in Wiskott-Aldrich syndrome: case report and literature review.
The case reported concerns a 29-year-old man who was seen because of clinical and laboratory features consistent with Wiskott-Aldrich Syndrome. While an infant he underwent splenectomy for thrombocytopenia. Evaluation revealed small platelets, abnormal immunoglobulin levels, impaired delayed hypersensitivity, and mildly reduced neutrophil chemotaxis. His response to vaccination with polyvalent pneumococcal vaccine was subnormal. Possible factors accounting for his long-term survival are discussed.
D000328:Adult; D001792:Blood Platelets / Q000473:pathology; D002634:Chemotaxis, Leukocyte; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000032:analysis; D008297:Male; D012882:Skin Tests; D013156:Splenectomy; D013921:Thrombocytopenia / Q000628:therapy; D014923:Wiskott-Aldrich Syndrome / Q000097:blood / Q000276:immunology*
D007136:Immunoglobulins