Mantle Cell Lymphoma Stage II

Mantle cell lymphoma stage II affects two or more groups of lymph nodes on the same side of the diaphragm. This rare blood cancer develops when abnormal B cells grow out of control in the lymphatic system, typically requiring treatment with a combination of therapies.

Table of contents

• What Is Mantle Cell Lymphoma?
• Understanding Stage II Disease
• Signs and Symptoms
• Diagnosis and Testing
• Treatment Approaches
• Outlook and Prognosis

What Is Mantle Cell Lymphoma?

Mantle cell lymphoma is a rare type of non-Hodgkin lymphoma, which is a cancer of the lymphatic system (the part of your body that helps fight infections)^[1]. The lymphatic system includes lymph nodes, spleen, bone marrow, and other organs that contain infection-fighting white blood cells called lymphocytes^[1].

This cancer develops from B cells (also called B lymphocytes), a specific type of white blood cell^[1]. The disease gets its name because the abnormal B cells usually develop in a part of your lymph nodes called the “mantle zone”^[1].

Mantle cell lymphoma is unusual compared to other lymphomas because it often has features of both fast-growing and slow-growing cancers^[1]. It typically grows quickly like aggressive lymphomas, but it can also behave more slowly in some patients^[4].

This type of lymphoma is rare. It makes up only about 5% to 6% of all non-Hodgkin lymphomas, with approximately 3,000 to 4,000 new cases diagnosed each year in the United States^[2][4]. The disease most commonly affects men over the age of 60, with men being diagnosed about three times more often than women^[2][4].

• Lymph nodes
• Spleen
• Bone marrow
• Liver
• Gastrointestinal tract
• Blood

Understanding Stage II Disease

When doctors diagnose mantle cell lymphoma, they determine its “stage” to understand how far the cancer has spread in your body. This information helps them plan the best treatment^[2].

Stage II mantle cell lymphoma means the cancer is found in two or more lymph nodes or groups of lymph nodes next to each other on the same side of the diaphragm (the muscle that separates your chest from your abdomen)^[2][11]. This is considered limited-stage disease^[12].

Stage II disease is relatively uncommon in mantle cell lymphoma. Approximately 5% to 15% of patients present with limited stage I or stage II disease^[16]. Most people with this type of lymphoma are diagnosed at more advanced stages (stage III or IV) because the cancer has already spread more widely by the time symptoms appear^[12].

Signs and Symptoms

Some people with mantle cell lymphoma have no obvious symptoms, which can delay diagnosis^[2]. When symptoms do occur, they can vary from person to person and may look like ordinary illnesses rather than cancer^[5].

The most common symptom is one or more painless swellings. These swellings are enlarged lymph nodes and typically appear in the neck, armpit, or groin^[7].

Other symptoms you might experience include:

• Heavy sweating at night (night sweats)
• Fever that comes and goes with no obvious cause
• Losing a lot of weight without trying (more than one-tenth of your total body weight)
• Fatigue (feeling very tired)
• Weakness
• Loss of appetite
• Nausea
• Belly pain or bloating

These symptoms are taken from multiple sources^[2][5][7].

Doctors call the group of night sweats, fever, and significant weight loss “B symptoms.” It is important to tell your doctor about any symptoms like this because they can affect your treatment plan and outlook^[7].

Mantle cell lymphoma can develop outside the lymph nodes. The symptoms you have depend on where it grows. For example, if it grows in your stomach or bowel, it might cause pain, diarrhea, or sickness. If it affects your bone marrow, it can cause anemia (low red blood cell count), bruising, or bleeding problems. If it affects your spleen, you might feel full very quickly when you eat or have discomfort behind your ribs^[7].

Diagnosis and Testing

Diagnosing mantle cell lymphoma often begins with a physical exam. Your healthcare provider will check for swollen lymph nodes in your neck, underarms, and groin, and will also check for a swollen spleen or liver^[3].

Healthcare providers usually suspect mantle cell lymphoma after routine blood work shows a high lymphocyte count (an elevated number of white blood cells). If your lab results are abnormal, your provider will do an exam and ask you to describe your symptoms^[5].

Blood tests can sometimes show if lymphoma cells are present in your blood. Blood tests also measure levels of lactate dehydrogenase (LDH), which is often higher in people with lymphoma^[3]. You may also have a complete blood count (CBC), comprehensive metabolic panel (CMP), or uric acid level test^[5].

The main test to diagnose mantle cell lymphoma is a biopsy, which is a procedure to remove a sample of tissue for testing in a lab^[3]. A doctor removes a sample from the affected area, often removing part or all of a swollen lymph node^[7]. The sample goes to a lab where a specialist looks at it under a microscope to check for cancer cells^[3].

In the lab, tests may show whether you have mantle cell lymphoma. The cells are checked for specific markers, including CD5 and CD20, which are proteins found on the surface of the abnormal cells. The cells are also tested for overproduction of a protein called cyclin D1, which is characteristic of mantle cell lymphoma^[4][12].

Your doctor may also recommend a bone marrow biopsy, which involves collecting cells from the bone marrow (the soft matter inside bones where blood cells are made)^[3]. Most often, samples are taken from the hip bone. In mantle cell lymphoma, this procedure is done to help determine the cancer’s extent^[3].

Imaging tests make pictures of your body and can show the location and extent of mantle cell lymphoma^[3]. Tests might include:

• CT scan (computed tomography scan)
• PET scan (positron emission tomography scan)
• MRI scan (magnetic resonance imaging)

You might have an endoscopy if your doctor thinks your lymphoma might be affecting your digestive system. This is when the doctor passes a thin tube through your mouth or your back passage to look at your digestive system and take biopsies of any abnormal looking tissue^[7].

Treatment Approaches

The type of treatment selected for a patient with stage II mantle cell lymphoma depends on multiple factors, including your age, overall health, and whether you have symptoms^[2][11].

Most often, you will receive chemotherapy (drugs that kill cancer cells) combined with immunotherapy (drugs that help your immune system fight cancer)^[2]. Your doctor may also use radiation therapy, targeted therapy, and stem cell transplants. Surgery usually is not helpful for mantle cell lymphoma^[2].

Chemotherapy drugs travel through your bloodstream to kill cancer cells wherever they are found^[2]. You will usually get a combination of drugs. Common chemotherapy approaches include:

• R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone)
• Bendamustine combined with rituximab
• Nordic protocol chemotherapy regimen (alternating cycles of different drug combinations)

These treatment approaches are described in multiple sources^[7][15].

Immunotherapy drugs use the natural disease-fighting power of your immune system. Some drugs attach to the surface of cancer cells so it is easier for your immune system to find and kill them. Others boost the natural ability of your immune system cells to fight cancer. Immunotherapy is often used with chemotherapy^[2].

Rituximab (Rituxan) is a drug that attaches to proteins on the surface of lymphoma cells and helps your immune system destroy them^[15]. It is commonly used as part of treatment combinations for mantle cell lymphoma.

Radiation therapy uses X-rays to kill cancer cells and shrink tumors^[2]. It targets a very specific area of your body, usually your lymph nodes. The treatment is quick and painless, but side effects like skin reactions and tiredness are common. You will usually get several small doses over the course of a few weeks. Doctors often use radiation when your cancer does not respond to chemotherapy^[2].

For younger, medically fit patients, doctors may recommend more intensive treatment approaches. These include combination chemotherapy followed by autologous stem cell transplantation (in which patients receive their own stem cells)^[15][16]. This approach is used to prolong remission (a period when there are no signs of cancer)^[15].

For older or less fit patients, less intensive chemotherapy followed by a prolonged course of rituximab alone (called maintenance therapy) is often recommended^[15].

Outlook and Prognosis

Mantle cell lymphoma is considered a difficult cancer to treat, but tremendous progress has been made in the discovery of new treatments for the disease^[15]. There is no cure for mantle cell lymphoma, but treatment can manage your symptoms and lead to long periods of remission^[5].

With mantle cell lymphoma, you might have periods of remission (when the cancer goes away or is controlled) followed by periods of recurrence (when the cancer comes back). This can happen several times. Treatment can lengthen the amount of time you are in remission^[5].

Most patients with aggressive mantle cell lymphoma have a median survival exceeding 8 to 10 years with current treatments^[12]. Some patients with a more slow-growing form of the disease can have a significantly better outlook, with a median survival exceeding 15 years^[12].

The outlook for individual patients varies greatly depending on many factors, including age, overall health, response to treatment, and specific characteristics of the cancer cells^[12].