Abstract
Asplenia and splenic hypoplasia refer to the complete or partial lack (or function) of splenic tissue. Asplenia can be congenital, functional (as in sickle cell anemia), or acquired after trauma or surgery. Congenital asplenia is a rare life-threatening condition, often presenting with pneumococcal sepsis. It may arise as part of situs abnormalities or result from a specific defect of spleen development. Most of the reported cases are familial and the mode of inheritance is usually autosomal dominant. In addition, a wide range of diseases can affect the function of the spleen, including metabolic, autoimmune, infectious and hematological disorders. The spleen plays an important role in the defense against pathogens and immune-homeostasis. Its rich and diverse population of immune cells and its anatomy enables optimal surveillance and phagocytosis of circulating blood elements. Moreover, the spleen is crucial in humoral response to polysaccharides, including antigens of encapsulated bacteria. For patients suspected to have splenic impairment, it is important to quantify the splenic function in order to assess the risk of developing overwhelming post-splenectomy syndrome and thromboembolic events. The prevention and treatment of these complications are described in this chapter.
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