Nodal Marginal Zone B-Cell Lymphoma

Nodal marginal zone B-cell lymphoma is a rare, slow-growing type of blood cancer that develops when white blood cells called B lymphocytes grow out of control in the lymph nodes.

Table of contents

• What is nodal marginal zone B-cell lymphoma?
• Parts of the body affected
• Who gets this condition?
• Causes and risk factors
• Symptoms
• Diagnosis and testing
• Treatment options
• Outlook and transformation

What is nodal marginal zone B-cell lymphoma?

Nodal marginal zone B-cell lymphoma is a type of blood cancer that develops when white blood cells called lymphocytes (cells that are part of your immune system and help fight infections) grow out of control.^[1] More specifically, it develops from B lymphocytes (also called B cells), one of two types of lymphocytes in the body.^[1]

This condition is grouped as a non-Hodgkin lymphoma (a broad category of blood cancers affecting the lymphatic system).^[1] It belongs to a group of related conditions called marginal zone lymphomas, which are slow-growing (also called low-grade or indolent) types of non-Hodgkin lymphoma.^[1][5] These lymphomas get their name because they develop in a particular region found at the edge of normal collections of lymphocytes called the marginal zone.^[1]

There are three types of marginal zone lymphoma: nodal marginal zone lymphoma (which affects lymph nodes), extranodal marginal zone lymphoma of mucosa-associated lymphatic tissue (MALT lymphoma, which can affect the stomach, lungs, skin and other organs), and splenic marginal zone lymphoma (which affects the spleen).^[3][5]

• Lymph nodes
• Bone marrow
• Blood

Parts of the body affected

Nodal marginal zone lymphoma usually develops in lymph nodes throughout the body.^[5] Lymph nodes are small, bean-shaped organs that are part of your lymphatic system and help filter out harmful substances. This condition can also affect the bone marrow (the soft tissue inside bones where blood cells are made) and blood.^[3][5]

Who gets this condition?

Nodal marginal zone lymphoma is a rare condition. Marginal zone lymphomas as a group usually affect people aged 60 and older, and they tend to be more common in men than in women.^[3]

Causes and risk factors

The exact causes of nodal marginal zone lymphoma are not fully understood. Research shows that in general, people with marginal zone lymphomas may have a family history of lymphoma, frequent infections, or autoimmune diseases (conditions where the immune system attacks the body’s own tissues).^[3]

Nodal marginal zone lymphoma specifically has been associated with hepatitis C virus (HCV) infection.^[3][5] If you have a hepatitis C infection, you will be treated with antiviral drugs before starting cancer treatment, as treating the infection first can sometimes reduce the symptoms of the lymphoma.^[5]

Risk factors for developing this condition include having a family history of lymphoma and having certain infections and autoimmune disorders.^[3]

Symptoms

Nodal marginal zone lymphoma typically grows very slowly. You may have this condition without experiencing any symptoms at all.^[3] When symptoms do occur, they may include:^[3]

• Fever
• Night sweats
• Unexplained weight loss

Because this is a slow-growing disease, many people do not develop symptoms right away, which is why regular monitoring and checkups are important.

Diagnosis and testing

Healthcare providers diagnose nodal marginal zone lymphoma by asking questions about your symptoms, your medical history, and your family medical history.^[3] The diagnosis process often begins with a physical exam that checks for swollen lymph nodes in the neck, underarms, and groin, as well as an enlarged spleen.^[10]

Several tests may be used to confirm the diagnosis:^[3][10]

• Blood tests: These can show whether lymphoma cells are present and check for infections like hepatitis C. Specific blood tests may include a complete blood count (CBC), lactate dehydrogenase (LDH) test, beta-2 microglobulin (a marker for blood cancers), and liver and kidney function tests.
• Imaging tests: These make pictures of the body to show the location and extent of the lymphoma. Tests might include ultrasound, MRI (magnetic resonance imaging), CT (computed tomography) scans, and PET (positron emission tomography) scans.
• Lymph node biopsy: This procedure involves removing all or part of a lymph node to look for cancer cells. The sample goes to a laboratory for testing to determine whether you have marginal zone lymphoma.
• Bone marrow aspiration and biopsy: These procedures collect cells from the bone marrow for testing. A needle is used to draw a sample of the fluid (aspiration) and solid tissue (biopsy), typically from the hip bone. This helps determine the extent of the cancer.

Treatment options

Treatment for nodal marginal zone lymphoma depends on several factors, including how fast the disease is progressing, whether you have symptoms, your age, and your overall health.^[5] Your healthcare team will work with you to develop a treatment plan tailored to your needs.

Antiviral treatment for hepatitis C: If you have a hepatitis C virus (HCV) infection, you will be treated with antiviral drugs before starting cancer treatment. Treating the HCV infection first can sometimes reduce the symptoms of nodal marginal zone lymphoma, and you may not need additional treatment.^[5]

Watchful waiting (also called active surveillance): If the lymphoma develops slowly and doesn’t cause symptoms, your healthcare team may recommend careful monitoring instead of immediate treatment.^[5][11] Your overall health and disease will be monitored through regular checkup visits and various tests. Active treatment is started when symptoms appear or there are signs that the disease is progressing more quickly.^[5]

Radiation therapy: This treatment uses high-energy rays or particles to destroy cancer cells. It is commonly used to treat nodal marginal zone lymphoma that is in specific areas of the body.^[5]

Targeted therapy: These drugs target specific molecules (such as proteins) on cancer cells or inside them. They stop the growth and spread of cancer cells while limiting harm to normal cells.^[5] Targeted therapy can be used alone or in combination with chemotherapy.

The most common targeted therapy drug used is rituximab (Rituxan and similar versions called biosimilars). If the lymphoma comes back (relapses) after treatment or stops responding to treatment (called refractory disease), other targeted therapy drugs may be used, including ibrutinib (Imbruvica), zanubrutinib (Brukinsa), and lenalidomide (Revlimid).^[5]

Chemotherapy: This treatment uses drugs to destroy cancer cells. It is often combined with targeted therapy drugs to treat nodal marginal zone lymphoma. Chemotherapy may also be used alone or in combination with radiation therapy.^[5]

The most common drug combinations used are:^[5]

• BR – bendamustine (Treanda, Benvyon, Esamuze) and rituximab
• R-CHOP – rituximab, cyclophosphamide (Procytox), doxorubicin, vincristine and prednisone
• R-CVP – rituximab, cyclophosphamide, vincristine and prednisone
• Chlorambucil (Leukeran) and rituximab
• Cyclophosphamide and rituximab

Stem cell transplant: A stem cell transplant (also called bone marrow transplant) may be considered in some cases, particularly if the disease returns after other treatments.^[5]

Outlook and transformation

Nodal marginal zone lymphoma is typically a slow-growing disease. Healthcare providers can treat and sometimes cure some cases of this condition.^[3]

One important aspect to be aware of is that sometimes nodal marginal zone lymphoma can change (transform) into an aggressive (fast-growing) type of non-Hodgkin lymphoma called diffuse large B-cell lymphoma (DLBCL).^[5] If this transformation occurs, the disease is treated like diffuse large B-cell lymphoma, which requires more intensive treatment approaches.

Regular follow-up care with your healthcare team is important to monitor the disease, manage any side effects of treatment, and watch for signs of transformation or relapse.