The syndrome of post-splenectomy fulminant sepsis. Case report and review of the literature.

Three years after splenectomy, a middle-aged woman had two separate episodes of fulminant bacterial sepsis. She recovered each time with prompt and appropriate treatment. Her immunologic system was examined and found to be normal with respect to antibody formation against diphtheria and tetanus toxoid, granulocyte killing of staphylococci and serum opsonizing activity. Granulocytes, bursa-equivalent (B) and thymus-dependent (T) lymphocytes and serum immunoglobulins were quantitatively normal. She produced antibody against subcutaneously injected polyvalent pneumococcal vaccine. She demonstrated cutaneous anergy. The literature on this syndrome was reviewed in an attempt to ascertain why hyposplenic patients are subject of fulminant bacterial, chiefly pneumococcal, sepsis. The probably explanation is the delayed production of antibodies against the phagocytic-resistant capsule of certain bacteria which the host had not previously encountered. The incidence of this syndrome appears to be on the order of 0.5 to 1.0%/year for splenectomized older children and adults. As the syndrome is seen chiefly in splenectomized or othewise hyposplenic patients, a causal relationship seems to exist.