Congenital

 Congenital asplenia is rare. There are two distinct types of genetic disorders:
heterotaxy syndrome[1] and isolated congenital asplenia.[2]
 polysplenia

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
 After splenectomy with the goal of arresting the progression of cancers (Chronic
lymphocytic leukemia,[3] Hodgkin's Disease (starting in the 1970s[4]), non-Hodgkin's
Lymphoma[5])
 Due to underlying diseases that destroy the spleen (autosplenectomy), e.g. sickle-cell
disease.
 Celiac disease: unknown physiopathology.[6] In a 1970 study,[7] it was the second
most common cause of abnormalities of red blood cells linked to hyposplenism, after
surgical splenectomy.
 Asplenia is a form of immunodeficiency, increasing the risk of sepsis from
polysaccharide encapsulated bacteria,[14] and can result in overwhelming post
splenectomy infection (OPSI), often fatal within a few hours. In particular, patients
are at risk from Streptococcus pneumoniae, Haemophilus influenzae, and
meningococcus.[14] The risk is elevated as much as 350–fold.[15]
 The increased risk of infection is due to inability to clear opsonised bacteria from
circulating blood. There is also a deficiency of T-cell independent antibodies, such as
those reactive to the polysaccharide capsule of Streptococcus pneumoniae.[16]
 The risk to asplenic patients has been expressed as equivalent to an adult dying in a
road traffic accident (in every 100 people without spleens, 1 to 5 would develop a
severe infection per decade) (reference UK Splenectomy Trust Advice)—hence
sensible precautions are advisable.[17] Increased platelet counts can be seen in
individuals without a functioning spleen.