Concept

Asplenia

Summary
Asplenia refers to the absence of normal spleen function and is associated with some serious infection risks. Hyposplenism is used to describe reduced ('hypo-') splenic functioning, but not as severely affected as with asplenism. Functional asplenia occurs when splenic tissue is present but does not work well (e.g. sickle-cell disease, polysplenia) -such patients are managed as if asplenic-, while in anatomic asplenia, the spleen itself is absent. Congenital asplenia is rare. There are two distinct types of genetic disorders: heterotaxy syndrome and isolated congenital asplenia. polysplenia Acquired asplenia occurs for several reasons: Following splenectomy due to splenic rupture from trauma or because of tumor After splenectomy with the goal of interfering with splenic function, as a treatment for diseases (e.g. idiopathic thrombocytopenic purpura, thalassemia, spherocytosis), in which the spleen's usual activity exacerbates the disease After splenectomy with the goal of arresting the progression of cancers (Chronic lymphocytic leukemia, Hodgkin's disease (starting in the 1970s), non-Hodgkin lymphoma) Due to underlying diseases that destroy the spleen (autosplenectomy), e.g. sickle-cell disease. Celiac disease: unknown physiopathology. In a 1970 study, it was the second most common cause of abnormalities of red blood cells linked to hyposplenism, after surgical splenectomy. Functional asplenia can occur when patients with metabolic or haematological disorders have their splenic tissue organisation altered. This can lead to results similar to those seen in patients who have undergone a splenectomy e.g. becoming infected with encapsulated bacteria such as Haemophilus influenzae, Streptococcus pneumoniae and Neisseria meningitidis. Patients who have some form of asplenia have an increased susceptibility to these encapsulated bacterial infections mainly because they lack IgM memory B cells and their non-adherence to polysaccharide vaccines. Furthermore, there is a deficiency of other splenic cells e.g. splenic macrophages.
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