Primary Immunodeficiency and Lymphoma Risk: What To Know

Primary immunodeficiency (PI), also called inborn errors of immunity (IEI), doesn’t just affect your ability to fight infections. PI also raises your risk of developing a blood cancer called lymphoma.

Studies estimate that people with PI have between a 4 percent and 25 percent chance of developing cancer. Lymphoma, especially B-cell non-Hodgkin lymphoma (NHL), is the most common cancer in PI.

We’ll cover what’s known about the risk of lymphoma with PI and how it develops. We’ll also discuss symptoms of lymphoma to look out for and common treatments.

Types of PI and Lymphoma Risk

Researchers aren’t quite sure why some people with PI develop lymphoma, while others don’t. They believe it may be due to:

• Trouble making antibodies
• Difficulty fixing damaged DNA
• Ongoing inflammation

Studies show that two types of PI are linked to most cancers in people with PI. These are common variable immunodeficiency (CVID) and ataxia-telangiectasia (AT).

Compared to the general population, people with CVID are 12 times as likely to develop NHL. Studies of people with AT show that cancer occurs in around 25 percent of cases. NHL and Hodgkin lymphoma (HL) are the most common.

Other types of PI are also associated with lymphoma, but at lower rates.

Severe combined immunodeficiency (SCID) is associated with EBV infection, which may lead to lymphoma. Having SCID raises your risk of HL and Burkitt lymphoma.

X-linked lymphoproliferative disease (XLP), also called Duncan disease, is associated with EBV infection. In XLP, NK cells don’t work as they should, putting you at risk for lymphoma. This type of PI is associated with a higher risk of NHL and HL.

Autoimmune lymphoproliferative syndrome (ALPS) develops when your body makes too many immune cells called lymphocytes. ALPS is associated with lymphoma.

Age at PI Diagnosis and Lymphoma Risk

Your age when you were diagnosed with PI can also affect your lymphoma risk. Lymphoma is more common in teenagers and young adults with Wiskott-Aldrich syndrome, a rare inherited disorder that affects the immune system and blood clotting.

Adults diagnosed with CVID have three to four times the cancer risk of people diagnosed before age 16. NHL and other types of cancer are usually found in people ages 30 and older with CVID.

Does Having a PI Mean You’ll Develop Lymphoma?

Studies show that people with PI conditions are more likely to develop lymphoma. However, this doesn’t mean that having PI means you will develop this blood cancer.

Many people live cancer-free lives with PI, even if their type of PI has a higher rate of lymphoma. Your doctor or PI specialist can help you better understand your individual cancer risk.

Types of Lymphoma Seen in Primary Immunodeficiency

PI raises your chances of developing certain types of lymphoma. Among people with PI, the most common cancers are NHL and HL. Overall, these two cancers account for 48.6 percent of cancer cases seen in PI.

Other types of lymphoma seen in PI include:

• Burkitt lymphoma
• Diffuse large B-cell lymphoma
• EBV-associated lymphoma
• T-cell lymphoma
• T-cell lymphoblastic lymphoma

The type of lymphoma depends on the immune cells affected. Some are T-cell lymphomas, and others are B-cell cancers. NHL can affect T cells, B cells, or NK cells, whereas HL typically only affects B cells.

Doctors diagnose the type of lymphoma based on how the cancer cells look under a microscope. Knowing the type you have will help guide your treatment plan.

Symptoms of Lymphoma To Watch For

If you have PI, it’s a good idea to know what lymphoma symptoms to watch for.

One of the first noticeable symptoms is swelling in your lymph nodes. You’ll feel small, painless lumps, usually in your neck, groin, and armpits.

Other lymphoma symptoms may include:

• Night sweats
• Fatigue
• Unexplained fever above 103 degrees Fahrenheit lasting more than two days
• Chest pain and trouble catching your breath
• Abdominal pain and swelling
• Losing weight without trying

You may hear certain lymphoma symptoms called B symptoms. B symptoms refer to the combination of fever, night sweats, and weight loss, which can occur with HL and NHL.

If you notice any of these symptoms, be sure to tell your doctor. They can run some tests to look for lymphoma and rule out other possible causes.

Lymphoma Is Treatable

Doctors consider lymphoma one of the most treatable cancers. With the right treatment plan, many people go into remission, and some may be cured.

Some types of lymphoma grow slowly over time. You may need lifelong treatment to control the cancer and stop it from spreading.

Luckily, doctors and researchers are always finding new and better ways to treat lymphoma. PI conditions are rare, so researchers are still learning the best way to treat lymphoma in people with immune system disorders.

Can Lymphoma Be Prevented?

Unfortunately, there’s no way to guarantee you’ll never get lymphoma with PI. If you’re concerned about your risk of lymphoma with PI, be sure to talk to your PI specialist. You can ask whether you’ll need regular screenings with blood tests.

If your specialist thinks you may have lymphoma, they can run specific tests. These tests may check for inflammation and specialized proteins that may be seen in higher amounts with lymphoma.

Doctors recommend that everyone with PI get all recommended cancer screenings, just like people with healthy immune systems. This includes screenings for cancers linked to age, such as breast, colon, and prostate cancers.

Steps To Lower Your Risk

You can take extra steps to help lower your risk of cancer in general. Many of these same steps will help you stay healthy as well.

Overall, doctors recommend:

• Limiting unnecessary X-rays or scans that use radiation, especially if you have AT or CVID
• Eating a healthy diet
• Limiting alcohol and not smoking

Having PI doesn’t mean you’ll develop lymphoma. Knowing the possible symptoms and keeping up with recommended health screenings can help doctors find problems early. Talk to your healthcare team about your personal risk and any symptoms you notice.

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