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Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue, or MALT lymphoma, is a slow-growing type of non-Hodgkin lymphoma that develops in the lymphoid tissue outside the lymph nodes.

MALT lymphoma is the most common form of marginal zone lymphoma, though it¡¯s still rare. It¡¯s estimated that about 5,000 new cases of MALT lymphoma are diagnosed in the U.S. each year, according to lymphoma specialist

To learn more about this disease, we spoke with Chihara and radiation oncologist Here¡¯s what they shared.

Where does MALT lymphoma develop?

The stomach is the most common site for MALT lymphoma. This is known as gastric MALT lymphoma. It can also be found in other parts of the gastrointestinal tract.

¡°It¡¯s important to note that this is not a primary stomach cancer,¡± says Gunther. ¡°This is lymphoma that occurs in the stomach.¡±

Non-gastric MALT lymphoma most commonly affects the lungs, salivary glands, tissues around the eye and skin.

What are the symptoms of MALT lymphoma?

Symptoms can vary, depending on where the lymphoma is in the body. Some people may not have any symptoms, and the cancer is found incidentally when a doctor is testing for something else.

For gastric MALT lymphoma, symptoms often mimic those of an upset stomach and can include:

• abdominal pain
• bloating
• heartburn
• acid reflux

Non-gastric MALT lymphoma may cause the following symptoms, depending on where it occurs in the body:

• salivary gland: a mass you can feel or that shows up in imaging
• lung: cough or shortness of breath
• skin: a small bump on the skin that can resemble a bug bite or pimple

¡°If you have any of these symptoms for longer than a couple of weeks without improvement, or if the symptoms are worsening, you should see a doctor to be evaluated,¡± says Gunther.

What causes MALT lymphoma?

Some MALT lymphomas are associated with autoimmune disorders and infections, depending on where the lymphoma is located.

Helicobacter pylori (H. Pylori) infection, a common bacterial infection that can cause stomach ulcers, increases the risk for gastric MALT lymphoma. Many gastric MALT lymphoma patients will have an H. Pylori infection.

¡°The H. Pylori infection causes chronic inflammation in the stomach, which is thought to increase the risk of gastric MALT lymphoma,¡± says Chihara.?

MALT lymphoma on the skin may be linked to Borrelia burgdorferi infection, while the autoimmune disorder Sjogren¡¯s syndrome can increase the risk of MALT lymphoma in the salivary glands.

How is MALT lymphoma diagnosed?

Doctors need biopsies to diagnose MALT lymphoma. The type of biopsy depends on where the lymphoma has developed.

An interventional radiology image-guided biopsy may be used to diagnose MALT lymphoma in the salivary glands or lungs. A bronchoscopy can also be used for the lungs.

An endoscopy may be used to diagnose gastric MALT lymphoma.

After a MALT lymphoma diagnosis is made, doctors will try to determine the cancer stage.

¡°With MALT lymphoma, it¡¯s more common for patients to be diagnosed with early-stage disease, or localized disease that hasn¡¯t spread to distant lymph nodes or other parts of the body,¡± says Gunther.

The cancer¡¯s stage determines the treatment.?

How is MALT lymphoma treated?

Patients with gastric MALT lymphoma can be given antibiotics first to treat H. Pylori. Treating the infection often clears away the lymphoma.

If antibiotics don¡¯t work, patients with early-stage MALT lymphoma receive radiation therapy.

If MALT lymphoma is not localized, and if you¡¯re not experiencing any problems from the cancer, your doctor may choose to monitor you closely, rather than prescribing treatment.

¡°MALT lymphoma is often a very slow-growing disease, and people can live with it for long periods, sometimes without even knowing they have it,¡± says Gunther. ¡°MALT lymphoma is treatable in most cases. We don¡¯t want to cause the patient more harm with the treatment than the disease itself.¡±

For patients with progressive advanced-stage MALT lymphoma that is causing symptoms or trouble, there are systemic treatment options.

The first systemic treatment option is the monoclonal antibody rituximab or chemoimmunotherapy, which is a combination of chemotherapy and immunotherapy. You may be given a chemotherapy drug, such as CHOP or bendamustine plus rituximab.

Recurrent MALT lymphoma may be treated with oral drugs, such as BTK inhibitors ibrutinib and zanubrutinib, or the immunotherapy drug lenalidomide.

What new research is being done to advance MALT lymphoma treatment??

Gunther recently led a clinical trial looking at reducing the radiation dosage for gastric MALT lymphoma patients.

¡°In the past, the standard dose for radiation was at least two-and-a-half weeks of treatment. But because of the positive response to low-dose radiation in this type of cancer, we¡¯ve been looking to reduce the dose,¡± explains Gunther.

In the clinical trial, patients started with ultra-low-dose radiation, or just two days of treatment.

¡°We gave additional treatment ¡ª another 10 days of radiation ¡ª only to those patients who did not experience a complete response after the initial two days,¡± she says. ¡°This spared a majority of patients unnecessary treatment and side effects.¡±

Because MALT lymphoma is rare, more research is needed to help determine which treatments work best for patients with MALT lymphoma. But Chihara notes that MD Anderson continues to pursue new clinical trials with the goal of expanding treatment options for patients with MALT lymphoma.

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By the time I was diagnosed with stage IV double-hit B-cell lymphoma in early 2019, I was in really bad shape. Scans showed that the cancer was everywhere, and the lesions inside my skull were putting so much pressure on my brain that they were giving me double vision.

Fortunately, I chose MD Anderson for my lymphoma treatment.?

The first thing my doctors did was correct another hospital¡¯s misdiagnosis. Then, they hit the cancer hard and fast with intense chemotherapy. As a result, I¡¯ve been cancer-free now for four years and counting.

My double-hit lymphoma symptoms

My lymphoma symptoms started appearing in December 2018. First, I developed severe pain in my right hip. Then, I noticed a swollen lymph node on the right side of my groin and another one on the left. It wasn¡¯t long after that when I started experiencing double vision.

My local doctor thought the swollen lymph node was just a cyst. But he ordered an MRI to be sure before we scheduled surgery to remove it. That¡¯s when we discovered I had cancer. My initial diagnosis was regular B-cell lymphoma, a type of non-Hodgkin lymphoma. I didn¡¯t find out it was actually double-hit lymphoma until I got to MD Anderson.

How I got to MD Anderson

I¡¯m originally from northern Virginia. I moved to Houston in 2016. So, I¡¯d never heard of MD Anderson before my cancer diagnosis.

But my mom is a registered nurse, and my mother-in-law¡¯s brother is a doctor. So, when they heard I had cancer, both of them said, ¡°Get to MD Anderson. NOW.¡±

They told me that for something this complex, you have to go to the best. And MD Anderson is ranked No. 1 in the nation for cancer treatment. It¡¯s also practically in my backyard, so I had no excuse not to go.

My double-hit lymphoma diagnosis

Once I arrived at MD Anderson, I knew I was in the right place. Everyone there made such a positive impression on me. The level of concern I received just while making an appointment was light years ahead of everyone else. And the knowledge and speed with which my care team worked was so far above what I¡¯d experienced elsewhere, it blew my mind.?

At MD Anderson, the first thing lymphoma specialist did was order his own tests and scans. That¡¯s how we discovered I had a different kind of lymphoma than I¡¯d originally been told.?

Dr. Westin explained that ¡°double-hit¡± meant two different genes had switched places in my DNA. But it¡¯s a much more aggressive type of lymphoma than regular B-cell. It grows very fast and is harder to kill. So, if I¡¯d gotten the original treatment I¡¯d been prescribed, I wouldn¡¯t be here today. I¡¯m really glad I went to MD Anderson.?

My double-hit lymphoma treatment

The cancer was so advanced by the time I had my initial office visit with Dr. Westin that he scheduled my first round of chemotherapy for the very next day. He told me I needed a strong regimen called R-EPOCH. I also needed another type of chemotherapy to kill the cancer on my skull. R-EPOCH wouldn¡¯t be able to reach it through the blood-brain barrier.

Each chemotherapy treatment would require me to be admitted to the hospital. So, I spent 50 days over the next four months at MD Anderson.

The treatment was brutal. I dropped 20 pounds due to nausea alone. I also had some serious low points emotionally, when I was feeling so discouraged that I didn¡¯t know if I could go on. But I got a lot of support from my wife and the online lymphoma community. They told me not to give up because I was almost there.

Fortunately, I listened to them and kept going. And the treatment worked. The cancer went into remission, and it¡¯s been gone since June 2019.

My life today, four years into remission

I only come back to MD Anderson now for my annual check-up. When I think back to those early days of 2019 and remember how sick I was, I¡¯m truly amazed at how far I¡¯ve come. Back then, the cancer was?everywhere. My scans were black with it, over my entire body.

And then, just two months later, it was all gone, thanks to MD Anderson and some very strong chemotherapy. Even Dr. Westin was like, ¡°That¡¯s kind of insane.¡±

The promises I made to myself during double-hit lymphoma treatment

I promised myself two things while lying in my hospital bed that first day at MD Anderson:

1. Once I got done with chemo, I was not going to look like a guy who¡¯d just got done with chemo.
2. If I survived, I would find more ways to give back.?

I fulfilled my first promise by walking two miles every day during my treatments ¡ª even when I was hooked up to an IV with three different bags of chemo flowing into me. I also had a whole workout regimen that I did every single day. So, when I got done, I¡¯d met my goal: my body did not look like it had just gone through 20 rounds of chemo.

I¡¯m fulfilling my second promise now by supporting various veteran causes. I¡¯m a veteran, and this year, my U.S. Naval Academy classmates and I are bicycling across the country to our 40^th reunion to raise money for one of these charities. One leg of that journey is a 600-mile ride between Toledo and Annapolis.

Doing this is pretty far out of my comfort zone. But for me to be here and riding 80 miles a day at all is incredible, considering where I was four years ago. So, I am grateful. None of this would be possible if it weren¡¯t for MD Anderson.

or by calling 1-877-632-6789.

Central nervous system lymphoma, or CNS lymphoma, is an aggressive, rare subtype of non-Hodgkin lymphoma. It most often occurs when cancerous cells form in the lymph tissue of the brain and/or spinal cord.

To learn more about central nervous system lymphoma, we spoke with , director of our Lymphoma Clinical Research Program.

Where does CNS lymphoma occur within the body?

It¡¯s more common to see CNS lymphoma in the brain than in the spine, but sometimes it can be found in both. CNS lymphoma can be located only in the spine, but it¡¯s extremely rare. Additionally, this lymphoma can occasionally be found in the fluid that surrounds the brain and spine.

Primary CNS lymphoma occurs when cancer starts within the central nervous system and there¡¯s no prior history of lymphoma anywhere else in the body. If lymphoma was found initially somewhere else in the body as well as in the central nervous system, it¡¯s considered the second incidence of lymphoma, or secondary CNS lymphoma.

What are common symptoms of CNS lymphoma?

Common first signs of primary CNS lymphoma could include a lesion in the brain, along with one or more of the following:

• confusion
• weakness
• difficulty speaking
• headaches

Secondary CNS lymphoma symptoms are similar. If a person is diagnosed with lymphoma and develops new symptoms related to their brain or spine, that could be related to lymphoma. If someone was diagnosed and treated for lymphoma previously and now has a brain lesion that a biopsy revealed to be lymphoma, it¡¯s considered a secondary CNS lymphoma.?

How is CNS lymphoma diagnosed?

There are three main types of tests used to help diagnose CNS lymphoma: imaging, biopsy and lumbar puncture.

• Imaging: This can include a CT scan or MRI. An MRI is usually preferred because it provides higher resolution, allowing a doctor to see more of the anatomy of the brain and identify differences between normal tissue and something abnormal. Often, imaging just suggests that there is a problem or suspicion of cancer. Because an MRI can detect other things in the brain that may look similar to lymphoma, doctors need additional testing to confirm cancer.
• Biopsy: Performing a biopsy on the brain can be difficult, depending on where the lesion is located. But a biopsy is critical to allow pathologists to determine if it¡¯s cancer. The biopsy results eliminate uncertainty, which allows the doctor to provide a diagnosis confidently.
• Lumbar puncture: During this procedure, also known as a spinal tap, a needle is inserted into the lower back to collect fluid samples below where the spinal cord ends. This is the same fluid that bathes the spine and brain. A lumbar puncture is commonly used to see if there is evidence of cancer cells in the fluid. In some cases, it can be used instead of a biopsy.

What causes CNS lymphoma?

Uncontrolled HIV infection can increase the chances of developing CNS lymphoma, but more effective HIV therapies have made this less of an issue recently.

The risk for CNS lymphoma increases if a person has a suppressed immune system due to other autoimmune conditions or viruses, such as Epstein-Barr virus.

Scientists don¡¯t yet know the cause of CNS lymphoma, so there aren¡¯t any actions you can take to avoid developing it. However, it does not appear to be hereditary. That means you don¡¯t have to worry about getting it from a relative; it¡¯s more sporadic.

How is CNS lymphoma treated?

One challenge of treating CNS lymphoma is the blood-brain barrier, which protects our brain from potentially harmful chemicals.

Initial treatment for CNS lymphoma is usually the chemotherapy drug methotrexate. Its chemical structure enables it to get past the blood-brain barrier.

Radiation therapy can also be used to treat CNS lymphoma as an additional treatment beyond chemotherapy, or as a backup treatment if chemotherapy doesn¡¯t work. At MD Anderson, doctors use sophisticated techniques to target radiation therapy precisely and converge on the intended point. Essentially, a patient can get low-dose radiation delivered to a specific part of the brain from many different directions. Or doctors can deliver high-dose radiation treatment to a specific part of the brain to avoid or minimize side effects to the rest of the brain.

Some patients may have consolidation therapy, which is given after initial treatment to kill any cancerous lymphoma cells left behind.

If a patient responds to initial treatment and they¡¯re relatively fit, they may undergo radiation or an autologous stem cell transplant to increase the effectiveness of the initial treatment and try to reduce the chances of recurrence.???

What new research is being done to advance CNS lymphoma treatment?

At MD Anderson, we¡¯ve had several targeted therapy clinical trials specifically focused on treating CNS lymphoma. We completed a study last year that used a combination of the drugs ibrutinib and nivolumab to treat patients with CNS lymphoma.

The targeted therapies that we¡¯ve seen get through the blood-brain barrier have great potential, both alone and in combination with other therapies.

We¡¯ve also been researching immunotherapies, including CAR T cell therapy and bispecific antibodies, which do a good job of getting the immune system to fight back against cancer cells in the body. These immunotherapies show great promise.

Is CNS lymphoma curable?

It depends on the general health of the patient. Sometimes this disease can show up in an elderly patient who has other medical issues. That¡¯s a different situation than a young patient who¡¯s fit and can tolerate high-dose treatment.

CNS lymphoma is a challenging disease to treat. However, some patients receive long-term benefits from standard treatments and will potentially be cured.?

But every situation is different. This is why it¡¯s so important to be treated at a place like MD Anderson that specializes in this type of cancer and can customize your treatment.

What else should we know about CNS lymphoma?

Symptoms of CNS lymphoma can change quickly since the cancer is located in the central nervous system, which controls our executive function for being able to walk, talk, eat and bathe. If a tumor within the brain grows by a centimeter or two, it can have a drastic impact on your ability to function.

Because CNS lymphoma is so rare and aggressive, the sooner you can get to a major cancer center like MD Anderson, the better. This is critical for an initial diagnosis or a recurrence. If CNS lymphoma is caught early, your body may respond better to treatment, or you may be able to join a clinical trial.

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Night sweats, persistent fevers and unexplained weight loss are three of the most common symptoms of lymphoma, whether they stem from Hodgkin lymphoma or non-Hodgkin lymphoma. But each one has to meet certain criteria to be considered a possible sign of blood cancer.

In adults, that means:?

• Night sweats must happen repeatedly, not be due to menopause-related hot flashes, and you should be drenched in sweat to the point that you have to get up and change your nightclothes and/or sheets at least once before morning.
• Weight loss must reflect 10% or more of your body weight, though you¡¯re not actively trying to lose it.
• Fever must be persistent, higher than 100.4 degrees Fahrenheit and not due to an underlying infection.?

¡°Lymphomas can be divided into two basic categories: aggressive and indolent,¡± explains lymphoma specialist ¡°Aggressive lymphomas grow and spread quickly, and can cause significant damage to tissue and organs. Because they are fast-growing, they are most commonly associated with what¡¯s known as ¡®B¡¯ symptoms. Indolent lymphomas, on the other hand, are slow-growing. They may not cause any symptoms at all, or cause symptoms that come and go over long periods of time.¡±

Here's how six of our patients knew they had lymphoma, in their own words.

Swollen lymph nodes

¡°I went to the doctor about a lump in my throat,¡± says Rob Moss, who was 49 when he was diagnosed with non-Hodgkin lymphoma. ¡°It grew and shrank several times. My doctor said it was probably a ¡®thyroglossal duct cyst,¡¯ or a growth on my thyroid. It turned out to be a swollen lymph node.¡±

Weight loss

¡°I lost 20 pounds in a few weeks, was extremely short of breath and was so tired I could barely cross a room without needing to sit down,¡± says Shelby Wade, who was 23 when she was diagnosed with B-cell lymphoma. ¡°I was also very hoarse and coughing a lot.¡±

Night sweats

¡°I experienced severe headaches, a consistent dry cough, weight loss and night sweats for about a month,¡± says Omar Dirani, who was 30 when he was diagnosed with non-Hodgkin lymphoma. ¡°I thought I was sweating a lot because of all the exercise I was doing.¡±

Cough

¡°I thought I had a cold or the flu,¡± says Jeff Johnson, who was 38 when he was diagnosed with Hodgkin lymphoma. ¡°But the cough that came with it never went away. I kept visiting our family physician and trying different medications, but nothing helped.¡±

Pain

¡°I was having back pains on the left side of my kidney area,¡± recalls Ann Sorhouse, who was 49 when she was diagnosed with non-Hodgkin lymphoma. ¡°But I was doing a lot of physical labor, so I attributed it to muscle strain.¡±

Itching

¡°I started having intense itching just a few months after I felt a lump on my collar bone,¡± recalls Mallory Parrish, who was 32 when she was diagnosed with Hodgkin lymphoma. ¡°At first, I thought it might be a yeast infection, but an over-the-counter anti-fungal treatment didn¡¯t help.¡±

When to see a doctor about your lymphoma symptoms

Ahmed recommends contacting a physician if your symptoms last more than two weeks without improvement.

¡°The more symptoms you have, the more concerned you should be,¡± she explains. ¡°If you have multiple B symptoms that last more than two weeks, see your doctor right away. If you only have one symptom, but it¡¯s inconsistent, just make your doctor aware of it.¡±

Lymphoma symptoms that you shouldn¡¯t ignore

Some lymphoma symptoms are considered so serious that they warrant an immediate trip to the emergency room. These include:

Spinal cord compression

This can manifest as back pain, leg weakness, bowel or bladder incontinence, vision problems, changes in mental status, or altered sensation in the areas of the leg and buttocks that would typically be in contact with a saddle.

Superior vena cava obstruction

This can appear as difficulty breathing, chest pain, face or neck swelling, or difficulty swallowing. It¡¯s usually due to a mass of clogged lymph nodes in the middle of the chest.

Tumorlysis

Caused by rapidly dying lymphoma cells, this condition can lead to heart arrhythmia, kidney problems and an overall sensation of just not feeling well. If you get blood work done, it may also show you have elevated potassium levels or white blood cell counts.

¡°Roughly 40% to 50% of patients with aggressive lymphomas show symptoms before their diagnosis,¡± notes Ahmed. ¡°But only about 10% or less of patients with indolent lymphoma do.¡±?

Lymphoma symptoms can occur with any type or stage of disease, but they¡¯re more commonly seen in fast-growing lymphomas and when the disease is more advanced. That¡¯s why it¡¯s important to get symptoms checked out quickly.

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