Splenic function: physiology and splenic hypofunction.

A wide variety of disorders can result in diminished splenic function. The pathophysiology appears to be clearly defined in some instances, such as congenital asplenia and disorders of splenic vascular obstruction or congestion. In others, such as the autoimmune and GI disorders, the mechanism remains poorly defined. Further research is needed. The hyposplenia which occurs in many of these disorders has been associated with an increased risk of life-threatening, overwhelming bacterial sepsis. In other instances, this complication has not been reported. This certainly should not be interpreted to mean that it cannot occur. The risk of septicemia in hyposplenic disorders is rarely above 10 to 15%. In disorders with minimal inhibition of splenic function, the incidence of sepsis would presumably be less than the 1.5% incidence following surgical splenectomy for trauma. Considering these data, a very large number of patients would have to become asplenic before it would be likely that one would develop sepsis. Furthermore, the lack of awareness of the possibility of hyposplenia-related sepsis in many of these disorders may cause such occurrences to go unrecognized. Finally, since the risk of sepsis is probably less in hyposplenic adults as compared to children, studies on adults may underestimate the incidence of this complication in children. Many of the disorders reported to cause hyposplenia in adults have not been noted to do so in children. In instances such as celiac disease, it may take many years for the complication to manifest so that it would be unlikely for a child to manifest hyposplenia during childhood. However, in other instances, not enough children have been studied to be confident that the hyposplenia and its associated risk of sepsis are not complications that occur in children. Hyposplenia-related bacterial septicemia is a catastrophic complication. If a patient develops a disorder that is potentially associated with hyposplenia, the patient should be observed for signs of asplenia in the peripheral blood. If the technique is available, quantitation of red cell pits should be performed. If not, other studies of splenic function such as radionuclide scans should be considered, depending on the incidence of hyposplenia in that particular disorder. If evidence of asplenia develops, pneumococcal vaccine should be administered, penicillin prophylaxis should be considered, significant febrile episodes should be managed aggressively, and probably most importantly, the patient and family should be carefully educated about this complication. Most deaths from hyposplenia-related septicemia are preventable.