Primary amyloidosis with diffuse splenic infiltration presenting as fulminant pneumococcal sepsis.

This case illustrates a unique clinical presentation of primary systemic amyloidosis, namely, overwhelming pneumococcal sepsis. Although there exists a well-established association between hyposplenism and overwhelming bacterial infection, amyloid replacement of the spleen as a primary cause of the hyposplenism has not been reported. Functional hyposplenism in regard to the effect of the spleen on erythrocytes has been reported in cases of diffuse splenic amyloid infiltration. The patient described had a fulminant course and associated disseminated intravascular coagulation, two clinical features more commonly seen in pneumococcemia occurring in asplenic patients as opposed to patients with normal splenic function. He had no predisposing factors, other than amyloid replacement of the spleen, to account for the development of overwhelming pneumococcemia. The immunologic function of the spleen in protection against overwhelming bacterial infection is briefly discussed.