Overwhelming sepsis in the adult variant of Wiskott-Aldrich syndrome.
Two brothers with a variant form of Wiskott-Aldrich syndrome suffered episodes of severe bacterial infection commencing in their fourth decade of life. One man died of overwhelming pneumococcal infection. This study emphasizes (1) the importance of recognizing the variant form of Wiskott-Aldrich syndrome in male adults with thrombocytopenia and (2) the treatment of these patients with prophylactic antibiotics.
D000328:Adult; D001424:Bacterial Infections / Q000150:complications* / Q000235:genetics / Q000276:immunology; D005260:Female; D006801:Humans; D008297:Male; D010375:Pedigree; D014923:Wiskott-Aldrich Syndrome / Q000150:complications* / Q000235:genetics / Q000276:immunology