Asplenia

Asplenia means you’re missing your spleen or your spleen doesn’t work correctly. Your spleen is a vital organ in your lymphatic system. It has several important jobs. One of its roles is to protect your body from germs, like bacteria.

If you don’t have a spleen, your body has trouble fighting certain bacteria. These bacteria have outer protective coverings. That makes them resistant to antibiotics. They include:

• Streptococcus pneumoniae
• Haemophilus influenzae
• Neisseria meningitidis

These bacteria can cause serious infections. They can lead to severe complications if you have asplenia. The risk of developing a life-threatening bacterial infection is higher in children with asplenia than in adults. But you can protect yourself and your loved ones by getting vaccinated and taking preventive antibiotics.

Types of asplenia

There are two main types of asplenia:

• Anatomic asplenia: This is when you don’t have a spleen. You may have had it surgically removed (splenectomy). Or it might have been missing from birth.
• Functional asplenia: This means you have a spleen, but it doesn’t work properly. This is often due to health conditions, like sickle cell disease or celiac disease.

What are the symptoms of asplenia?

Asplenia itself usually doesn’t cause any noticeable symptoms. It’s more important to look out for signs of an infection. An infection can be dangerous if you have asplenia. If you develop any of the following symptoms, reach out to your healthcare provider or go to the nearest emergency room right away:

• Fever
• Chills
• Rapid heart rate
• Fatigue
• Confusion
• Irritability

What causes asplenia?

The cause of asplenia depends on the type (anatomical or functional).

Anatomical asplenia

There are a couple reasons why you might not have a spleen:

• Surgical removal (splenectomy): A healthcare provider may need to remove your spleen due to trauma or injury. For example, a car accident may have caused a ruptured spleen. Rarely, providers use splenectomy to treat certain medical conditions.
• Congenital asplenia: This means you were born without a spleen. A genetic change (variation) in the RPSA gene (responsible for creating proteins) may lead to this condition. Sometimes, it occurs as part of a rare syndrome, like heterotaxy.

Functional asplenia

Certain medical conditions can damage your spleen. Examples include:

• Sickle cell disease
• Celiac disease
• Lupus
• Advanced HIV infection
• Liver disease

What are the complications of asplenia?

Asplenia increases your risk of developing serious bacterial infections, including:

• Pneumococcal disease
• Haemophilus influenzae type b (Hib)
• Meningococcal disease

These infections can lead to severe complications, including sepsis, organ failure and death.

Other complications of asplenia may include:

• Increased bleeding risk: Your spleen plays a role in storing platelets. You need platelets for blood clotting. Without a spleen, you have a slightly higher risk of bleeding.
• Autoimmune issues: Asplenia can upset the balance of your immune system. This can lead to autoimmune disorders. These conditions cause your body to attack its own tissues.
• Thrombosis: Asplenia can sometimes cause thrombosis. This occurs when blood clots form in your veins or arteries.

How doctors diagnose asplenia

Your healthcare provider will ask about any symptoms you have and your medical history. They’ll want to know if you have a biological family history of asplenia. Your provider will also perform a physical exam. The exam might not reveal signs of asplenia itself. But it can help your provider figure out if you have any underlying conditions or infections.

Tests that are used

Your provider will use imaging tests to see whether or not you have a spleen. If you do, the images can show the size and shape of your spleen. These tests may include:

• Ultrasound
• CT scan
• MRI scan

Your provider may also request a spleen scintigraphy. This is a specialized nuclear medicine imaging test. Your provider injects a small amount of radioactive material into your vein. If you have a spleen, it’ll absorb the material. If you don’t have a spleen, the material won’t be absorbed. Your provider will be able to see a “cold spot” on the scan.

Imaging tests can confirm if you don’t have a spleen. But they can’t tell how well your spleen is working. So, your provider may order a complete blood count (CBC). This test can show abnormalities in your red blood cells that may mean you have asplenia.

What are the treatment options for asplenia?

Asplenia itself isn’t treatable. Treatment involves preventing the serious infections that it can cause. Your healthcare provider will suggest vaccines and special antibiotics.

Vaccination

Your provider will recommend vaccines that help protect you from certain bacteria. These bacteria can cause life-threatening infections in people who aren’t vaccinated. These vaccines include:

• Pneumococcal vaccine
• Haemophilus influenzae type b vaccine
• Meningococcal polysaccharide vaccine

Depending on the reason for asplenia, you may get these vaccines as an infant. You can also get them later in life.

You also have an increased risk of developing influenza. So, your provider will recommend getting a seasonal flu vaccine.

Antibiotics

Your provider may recommend a prophylactic (preventive) antibiotic. These antibiotics help reduce your chances of developing a serious infection. You’ll take a regular, low dose of the medication. These antibiotics are especially useful for children younger than 5, since they have a higher likelihood of disease.

Your provider will also prescribe antibiotics to treat an infection. If you have any symptoms of infection — like fever, chills or fatigue — get medical care as soon as possible by going to the nearest emergency department. Early and aggressive antibiotic treatment is vital due to your increased risk of serious complications.

When should I see my healthcare provider if I have asplenia?

If you have asplenia, see your healthcare provider:

• If you have any symptoms of infection: A basic infection can be life-threatening. Early diagnosis and treatment with antibiotics are critical.
• Before certain medical procedures or dental work: Your provider might recommend antibiotics beforehand to reduce your infection risk.

What is the prognosis for asplenia?

Asplenia is a lifelong disease. But your prognosis (outlook) is generally good if you manage the condition properly. Talk to your healthcare provider. Stay up to date on their recommended vaccines and antibiotics. Make sure to seek medical care at the first sign of an infection. Without taking steps to reduce your risk of bacterial infections, your prognosis is poor.

How can I lower my risk of complications from asplenia?

In addition to vaccination and antibiotics, there are ways you can reduce your risk. These include:

• Maintain good hygiene: Wash your hands often and avoid close contact with people who are sick.
• Wear a medical alert bracelet: In case of an emergency, this informs healthcare providers about your condition.
• Early medical attention: Seek prompt medical care if you experience any symptoms of infection.