[Asplenism and hyposplenism as an immune deficiency syndrome]

The spleen has vital immunological functions (including the clearance of pathogenic microorganisms, the synthesis of antibodies and the provision of opsonins), which cannot be completely taken over by other organs in the case of hypo- or asplenism. Deficiency or absence of splenic activity, as it occurs for instance in sickle cell anemia, in congenital asplenia or after splenectomy, consequently entails complex immunological defects, which in their turn result in an increased susceptibility to severe bacteremia. Preventive measures, such as antibiotic prophylaxis, pneumococcal vaccination and detailed instruction of the patients and their families, are recommended in order to reduce the incidence of these life-threatening infections fatal to at least 1.4% of all splenectomized children.