Lymphoma — a cancer that starts in the lymphocytes (white blood cells) — isn’t a common type of cancer. Yet, people with Sjögren’s disease should be aware of their increased risk of lymphoma relative to the general population. Researchers used to believe that people with Sjögren’s were 44 times more likely to develop lymphoma than the general population, but they now know the risk is in the range of six to nine times higher.

So, what is lymphoma, and why are people with Sjögren’s more likely to get it? The higher risk of lymphoma in people with Sjögren’s disease starts in the immune system. In this article, we’ll explore the connections between Sjögren’s and lymphoma and tell you when it’s time to see a doctor.

Understanding Lymphoma

Lymphoma isn’t just one cancer, but rather a group of cancers affecting the lymphatic system. Your lymphatic system is part of the immune system — its vessels and glands help your body fight infections.

Although there are many lymphoma subtypes, there are just two main categories of lymphoma: Hodgkin lymphoma and non-Hodgkin lymphoma (NHL). If you have Sjögren’s disease, you’re at an increased risk for NHL specifically.

Non-Hodgkin lymphoma is far more common than Hodgkin lymphoma, though the two have similar symptoms. Non-Hodgkin lymphoma starts in either your B cells or T cells, which are two different types of lymphocytes. Because NHL affects immune system cells, it increases your risk for infections.

Abnormal white blood cells build up and form tumors, usually in the lymph nodes but sometimes in the thymus or spleen. Without cancer treatment, those tumors can metastasize (spread) throughout the body.

Causes and Risk Factors

Non-Hodgkin lymphoma develops when your immune system produces too many B or T cells. Rarely, it might develop when you produce too many of a third type of white blood cell called natural killer (NK) cells. Usually, an overproduction of white blood cells stems from an acquired genetic mutation (change), which is a gene change that occurs while you’re alive and not before you’re born.

Non-Hodgkin lymphoma risk factors include:

• Age over 60
• Male sex, according to Cleveland Clinic
• Family history of lymphoma
• Exposure to pesticides
• Autoimmune diseases — Including Sjögren’s disease, which carries the highest risk among autoimmune diseases, and rheumatoid arthritis
• Weakened immune system

Lymphoma Symptoms

Swollen lymph nodes in your armpits, groin, or neck that aren’t painful are the most common signs of lymphoma. However, swollen glands can have other more common causes.

Non-Hodgkin lymphoma can cause a wide variety of other symptoms, including:

• Persistent fever
• Night sweats
• Chest pain
• Fatigue (extreme tiredness that doesn’t improve when you rest)
• Enlargement of the belly and feeling full even when you haven’t eaten
• Unexplained weight loss

All of these symptoms have other possible explanations, so it’s important to see your doctor for an evaluation if you have these symptoms and they don’t go away on their own.

How Lymphoma Is Treated

Oncologists treat lymphoma based on its stage, the specific type of NHL you have, and your overall health at the time of diagnosis. If your non-Hodgkin lymphoma grows slowly and doesn’t cause many symptoms, your doctor may recommend active surveillance instead of immediate treatment.

The most common NHL treatment is chemotherapy, which involves drugs or medications that seek out and attack cancer cells. You may get chemotherapy on its own or alongside other cancer treatments like radiation therapy, targeted therapy, or stem cell transplantation.

Sjögren’s and Lymphoma Risk

The same lymphocytes involved in non-Hodgkin lymphoma are also active in Sjögren’s disease. B cells, or B lymphocytes, create proteins called antibodies that find, bind to, and destroy germs like viruses and bacteria. In people with Sjögren’s disease, overactive B cells cause inflammation. When B cells are highly active in this way, a person’s lymphoma risk is higher. T-cell lymphomas are far less common than B-cell lymphomas, which account for around 85 percent of all non-Hodgkin diagnoses.

Non-Hodgkin lymphoma alone has more than 70 subtypes, but the most common in people with Sjögren’s disease is a type of B-cell lymphoma called mucosa-associated lymphoid tissue (MALT) lymphoma. MALT lymphoma is slow-growing and usually diagnosed in early stages when it’s most treatable. It usually starts in the stomach, but in people with Sjögren’s disease, it often starts in the salivary glands. It can originate anywhere with a moist tissue lining, including the mouth, nose, and digestive tract.

Beyond the B cells, several factors can increase lymphoma risk in people with Sjögren’s disease. You’re more likely to develop non-Hodgkin lymphoma if you have:

• Raynaud’s phenomenon (poor circulation in your fingers, toes, and ears)
• Swollen salivary glands caused by overactive B cells
• Peripheral neuropathy (damage to nerves outside the brain and spinal cord)
• Petechiae or purpura (bleeding dots on the skin, most often on the lower legs)
• Lymph nodes that are almost always swollen
• Abnormal proteins in your blood

If you have Sjögren’s disease and more than three of these risk factors, your risk of non-Hodgkin lymphoma increases significantly. Although most people with Sjögren’s disease will never develop lymphoma, your healthcare provider or rheumatologist might recommend regular monitoring visits if multiple of these risk factors apply to you.

Monitoring Your Lymphoma Risk

Unlike other cancers, such as breast cancer or prostate cancer, there is no specific screening test for non-Hodgkin lymphoma. However, after evaluating your lymphoma risk, your doctor or rheumatologist might suggest frequent appointments to monitor you and check for lymphoma warning signs.

Warning Signs of Lymphoma in Sjögren’s

You might notice some warning signs of lymphoma on your own. Let your doctor know if:

• You feel like your lymph nodes or salivary glands are swollen.
• You often wake up drenched in sweat.
• You lose 10 percent of your body weight in six months without trying.

All of these are physical warning signs of lymphoma.

The Importance of Regular Checkups

Other lymphoma warning signs are more subtle, which is why regular checkups are so important. Your doctor examines you at these visits and investigates any symptoms you report, but they also use blood tests to look for specific proteins in your blood. They might also check your salivary glands for indicators of lymphoma.

If you have risk factors for lymphoma with Sjögren’s disease, your doctor might suggest scheduling checkups once every six to 12 months. The more risk factors and warning signs you have, the more often your doctor will want to see you.

Talk to Your Doctor

Talk to your doctor if you notice any symptoms beyond typical Sjögren’s symptoms, like dry eyes and dry mouth, especially those that might indicate lymphoma. You can also talk to your doctor about concerns about your lymphoma risk with Sjögren’s disease. Your doctor or Sjögren’s disease specialist can tell you more about your lymphoma risk and whether or not you should get regular checkups for lymphoma.

In some cases, doctors may prescribe a monoclonal antibody medication called rituximab to people with Sjögren’s disease who have a higher risk for lymphoma. This drug targets B cells and can help control inflammation. Although monoclonal antibodies can’t prevent lymphoma, some research suggests they can improve outcomes for people with non-Hodgkin lymphoma while also treating Sjögren’s.

Join the Conversation

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Have you been diagnosed with non-Hodgkin lymphoma? Let others know in the comments below.