The Syndrome of Asplenia, Pneumococcal Sepsis, and Disseminated Intravascular Coagulation

Abstract

Disseminated intravascular coagulation with Waterhouse-Friderichsen syndrome occurred during the course of fatal pneumococcal sepsis in a previously healthy woman. At postmortem examination the patient was found to have an atrophic spleen weighing 29 g. There have been four previously reported instances of fatal pneumococcal infection in which disseminated intravascular coagulation was documented by coagulation studies or pathologic findings. Waterhouse-Friderichsen syndrome was observed in each case. Three of the patients had undergone splenectomy, and the fourth had splenic atrophy demonstrated at autopsy. A number of additional reports of pneumococcal sepsis with Waterhouse-Friderichsen syndrome (but without documented disseminated intravascular coagulation) have been published. Almost all such patients have had atrophic spleens or no spleens. Possible pathogenetic mechanisms are discussed.