Fatal pneumococcal Waterhouse-Friderichsen syndrome

There have only been six deaths reported in the English literature due to disseminated intravascular coagulation and Waterhouse-Friderichsen syndrome complicating pneumococcemia in otherwise healthy persons with normal spleens. Four of these deaths occurred in children and two in adults. A case of fulminant pneumococcemia complicated by disseminated intravascular coagulation and Waterhouse-Friderichsen syndrome in an otherwise healthy adult with a normal spleen is presented. The case is typical of the course of fulminant pneumococcal sepsis and highlights some of the difficulties experienced by rural general practitioners and rural retrieval services. Mechanisms by which Pneumococcus can elicit fulminant sepsis are discussed, although there is no explanation as to why this may occur in the setting of normal splenic function.