Asplenia


Asplenia

Description, Causes and Risk Factors:

Asplenia refers to the absence of normal spleen function and is associated with some serious infection risks.

Asplenia

Asplenia is a syndrome of right isomerism or attempted bilateral right sidedness. Bilateral right-sidedness is manifest as bilateral trilobed lungs, bilateral right atrial appendages, transverse liver and absence of the spleen. Asplenia is usually associated with severe congenital heart disease including complete atrioventricular canal, transposition of the great arteries, anomalous pulmonary venous drainage and pulmonic or less commonly aortic stenosis or atresia. Because of the severity of the heart disease and the immune defect from absence of the spleen, the mortality (the quality or state of being mortal) among patients with asplenia is high. Intestinal malrotation and malfixation are present in essentially all patients with this disease.

It is estimated that the incidence of asplenia is low, approximately one in 10,000 to one in 20,000 live births. More males are affected with the condition than females. This disease also accounts for 1-3% of all congenital heart defects. Asplenia does not appear to occur more frequently in certain ethnic groups.

The causes of this disease could either be congenital or acquired. However, the cases of its occurrence due to genetic reasons are very rare.

Congenital:

    Congenital asplenia (rare) may be due to genetic disorders, (i.e. heterotaxy syndrome).

Acquired:

    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.

  • Due to underlying diseases that destroy the spleen (autosplenectomy), e.g. sickle-cell disease.

  • 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.

  • Trauma causing damage to the spleen can also cause this condition. Furthermore, sometimes a preexisting condition causes the spleen to lose its functionality. Diseases like sickle cell anemia can lead to this disease, for example. Sometimes, the spleen loses its functionality despite of its apparently healthy presence in the body as well, a condition known as functional asplenia. Furthermore, the condition obviously occurs in the case of splenectomy, which is the partial or complete surgical removal of the spleen. This surgical procedure is sometimes performed when the presence of a spleen is actually making a particular medical condition worse and when physicians are left with little choice but to go for its removal.

Other risk factors may include:

Alcoholism, amyloidosis, autoimmune hemolytic anemia, biliary cirrhosis, bone marrow transplantation, celiac disease, chronic active hepatitis, chronic graft versus host disease, chronic myelogenous leukemia, collagenous colitis, essential thrombocythemia, Graves' disease, hairy cell leukemia, Hashimoto's thyroiditis, hemangiosarcoma of the spleen, hemophilia, hematologic diseases, hereditary spherocytosis, Hodgkin's disease, idiopathic thrombocytopenic purpura, non-Hodgkin's lymphoma, ovarian carcinoma, portal hypertension, rheumatoid arthritis, right sided heart failure, sarcoidosis, Sj

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