What causes asplenia syndrome?
What causes asplenia syndrome?
Isolated congenital asplenia caused by mutations in the RPSA gene is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. In most cases, an affected person inherits the mutation from one affected parent .
What causes functional asplenia in sickle cell?
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.
Is asplenia and immunodeficiency?
Asplenia is a form of immunodeficiency, increasing the risk of sepsis from polysaccharide encapsulated bacteria, and can result in overwhelming post-splenectomy infection, often fatal within a few hours. In particular, patients are at risk from Streptococcus pneumoniae, Haemophilus influenzae, and meningococcus.
Does asplenia cause immunocompromised?
1,2 Patients who have had a splenectomy or have functional asplenia are immunocompromised and are at increased risk for severe and overwhelming bacterial infections, particularly from encapsulated bacteria.
Why does splenectomy cause infection?
Splenectomy may be followed by severe systemic infection because such surgery removes the splenic macrophages that filter and phagocytose bacteria and other bloodborne pathogens. Overwhelming post-splenectomy infection (OPSI), as this complication is called, is uncommon but has high mortality.
How does asplenia affect immunity?
Splenectomized patients have been shown to have low concentrations of IgM, decreased production of antibodies directed against pneumococci and Escherichia coli, and several defects in cellular immune function, including decreased numbers of T cells and a reduction in lymphocyte proliferative responses.
What qualifies as immunocompromised?
People are considered to be moderately or severely immunocompromised if they have: Been receiving active cancer treatment for tumors or cancers of the blood. Received an organ transplant and are taking medicine to suppress the immune system.
What causes enlarged liver and spleen?
Enlarged liver and spleen has a variety of causes including infections, blood disorders, liver disease, and cancers.
