Asplenia


Asplenia refers to the absence of normal spleen function and is associated with some serious infection risks. Hyposplenism is used to describe reduced splenic functioning, but not as severely affected as with asplenism.
Functional asplenia occurs when splenic tissue is present but does not work well -such patients are managed as if asplenic-, while in anatomic asplenia, the spleen itself is absent.

Causes

Congenital

Acquired asplenia occurs for several reasons:
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. This combined with the lack of B cells can provide an environment favourable for the development of bacterial infections.

Partial splenectomy and preservation of splenic function

In an effort to preserve some of the spleen's protective roles, attempts are now often made to preserve a small part of the spleen when performing either surgical subtotal splenectomy, or partial splenic embolization.
This may be particularly important in poorer countries where protective measures for patients with asplenia are not available.
However, it has been advised that preoperative vaccination is advisable until the remnant splenic tissue can reestablish its function.

Risks

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. The risk is elevated as much as 350-fold.
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.
The risk to asplenic patients has been expressed as equivalent to an adult dying in a road traffic accident —hence sensible precautions are advisable. Increased platelet counts can be seen in individuals without a functioning spleen.

Diagnosis

Diagnosis is confirmed by abdominal ultrasonography and detection of Howell-Jolly bodies in red blood cells.

Management

To minimise the risks associated with splenectomy, antibiotic and vaccination protocols have been established, but are often poorly adhered to by physicians and patients due to the complications resulting from antibiotic prophylaxis such as development of an overpopulation of Clostridium difficile in the intestinal tract.

Antibiotic prophylaxis

Because of the increased risk of infection, physicians administer oral antibiotics as prophylaxis after a surgical splenectomy, or starting at birth for congenital or functional asplenia.
Those with asplenia are also cautioned to start a full-dose course of antibiotics at the first onset of an upper or lower respiratory tract infection, or at the onset of any fever. Even with a course of antibiotics and even with a history of relevant vaccination, persons without a functional spleen are at risk for Overwhelming post-splenectomy infection.
In an emergency room or hospital setting, appropriate evaluation and treatment for an asplenic febrile patient should include a complete blood count with differential, blood culture with Gram stain, arterial blood gas analysis, chest x-ray, and consideration for lumbar puncture with CSF studies. None of these evaluations should delay the initiation of appropriate broad-spectrum intravenous antibiotics. The Surviving Sepsis Campaign guidelines state that antibiotics should be administered in a patient suspected of sepsis within 1 hour of presentation. Delay in starting antibiotics for any reason is associated with a poor outcome.

Vaccinations

It is suggested that splenectomized persons receive the following vaccinations, and ideally prior to planned splenectomy surgery:
In addition to the normal immunizations advised for the countries to be visited, Group A meningococcus should be included if visiting countries of particular risk. The non-conjugated Meningitis A and C vaccines usually used for this purpose give only 3 years coverage and provide less-effective long-term cover for Meningitis C than the conjugated form already mentioned.
Those lacking a functional spleen are at higher risk of contracting malaria, and succumbing to its effects. Travel to malarial areas will carry greater risks and is best avoided. Travellers should take the most appropriate anti-malarial prophylaxis medication and be extra vigilant over measures to prevent mosquito bites.
The pneumococcal vaccinations may not cover some of the other strains of pneumococcal bacteria present in other countries. Likewise their antibiotic resistance may also vary, requiring a different choice of stand-by antibiotic.

Additional measures