Malignant Lymphoid Neoplasm

Malignant lymphoid neoplasm, commonly known as lymphoma, is a group of blood cancers that develop when white blood cells in the lymphatic system grow out of control and spread to other parts of the body.

Table of contents

• What is lymphoma?
• Main types of lymphoma
• Common symptoms
• Causes and risk factors
• How lymphoma is diagnosed
• Treatment options
• Outlook and survival
• Living with lymphoma

What is lymphoma?

Lymphoma is a type of cancer that begins in the lymphatic system (the network of tissues, vessels and organs that help your body fight infection). When you have lymphoma, white blood cells called lymphocytes (cells that normally protect your body from germs and disease) change and start to grow uncontrollably. These abnormal cells don’t die when they should, and they can build up to form tumors in the lymph nodes and other parts of your body.^[1][2]

The lymphatic system includes lymph nodes (small bean-shaped structures), the spleen, bone marrow, thymus, tonsils, and lymph vessels that carry lymph fluid throughout your body. Because lymph tissue is found throughout the body, lymphoma can start almost anywhere and spread to many different organs.^[1][2]

Lymphoma represents approximately 5% of all cancers. More than 82,000 people are diagnosed with lymphoma each year in the United States, making it one of the more common blood cancers.^[3][5]

Main types of lymphoma

There are two main categories of lymphoma, with more than 70 different subtypes. The classification depends on what the cancer cells look like under a microscope and which type of lymphocyte is affected.^[2][5]

Hodgkin lymphoma makes up about 10% of all lymphoma cases. It is identified by the presence of specific abnormal cells called Reed-Sternberg cells. This type usually begins in lymph nodes of the neck, chest, or under the arms, and often progresses in a predictable pattern from one group of lymph nodes to the next.^[1][3]

Non-Hodgkin lymphoma accounts for about 90% of all lymphoma cases. If Reed-Sternberg cells are not found, the lymphoma is classified as non-Hodgkin. This category includes many different subtypes. Some non-Hodgkin lymphomas grow slowly (called indolent or low-grade), while others grow quickly (called aggressive or high-grade).^[2][3]

Non-Hodgkin lymphoma typically affects people between ages 60 and 80, and is more common in men than in women. Hodgkin lymphoma may affect people between ages 20 to 39 and those over age 65.^[2][5]

Common symptoms

Many lymphoma symptoms are similar to symptoms of other, less serious illnesses. The most common sign is swollen lymph nodes that don’t go away after a few weeks. These swollen nodes are usually painless and can appear in your neck, armpits, groin, or other parts of your body.^[1][2]

Other symptoms may include:

• Fever that stays above 103 degrees Fahrenheit (39.5 degrees Celsius) for more than two days, or a fever that keeps coming back
• Drenching night sweats that are so intense you wake up with your pajamas and sheets soaking wet
• Unexplained weight loss, when you’ve lost 10% of your total body weight over six months without dieting or exercise
• Persistent fatigue, feeling very tired day after day even after getting enough sleep
• Shortness of breath, feeling as if you can’t get enough air in your lungs
• Itching skin
• Cough or chest pain

The combination of fever, unexplained weight loss, and night sweats is sometimes called “B symptoms” by doctors and indicates more advanced disease.^[2][5]

Some swollen lymph nodes located inside your body can press on organs and cause additional symptoms, such as pain in your chest, stomach area, or difficulty breathing.^[6]

Causes and risk factors

In most cases, doctors don’t know exactly what causes lymphoma. Like most cancers, lymphoma happens when cells undergo genetic changes that make them grow faster than they should and live longer than they should. These genetic changes usually occur spontaneously, without an identifiable cause.^[2]

However, certain factors can increase your risk of developing lymphoma:

• Viral infections: Having or having had viruses including HIV (human immunodeficiency virus), Epstein-Barr virus (which causes mononucleosis), hepatitis C, human T-cell lymphotropic virus, or human herpesvirus 8^[2][3]
• Weakened immune system: People who have had organ transplants and take immunosuppressant medications, or those with HIV/AIDS or genetic immunodeficiency disorders^[2][3]
• Autoimmune diseases: Conditions where your immune system accidentally attacks your body, such as rheumatoid arthritis, Sjögren’s syndrome, or inflammatory bowel disease^[2][3]
• Family history: Having a family member with lymphoma increases your risk slightly, though lymphoma is not typically inherited^[2]
• Age and gender: Risk varies by type, but generally men are slightly more likely to develop lymphoma than women^[2][5]
• Occupational exposure: Contact with herbicides, pesticides, or certain chemicals^[3]
• Tobacco use and obesity: These are major modifiable risk factors^[5]

How lymphoma is diagnosed

If your doctor suspects lymphoma, several tests will be done to confirm the diagnosis and determine what type you have.

Physical examination: Your doctor will check for swollen lymph nodes in your neck, armpits, and groin, and may check the size of your liver and spleen.^[2]

Biopsy: An open lymph node biopsy is the preferred method for diagnosing lymphoma. During this procedure, a doctor removes cells or tissue from a lymph node or other affected area, and a specialist examines them under a microscope to look for cancer cells. This is the most important test for confirming lymphoma.^[2][5]

Blood tests: Blood work checks your overall health, blood cell counts, liver and kidney function, and can detect signs of infection. For some types of lymphoma, blood tests can also measure specific substances produced by cancer cells.^[2][13]

Imaging tests: Several types of scans help doctors see where lymphoma is located in your body:

• CT (computed tomography) scan: Creates detailed cross-sectional images of your body
• PET (positron emission tomography) scan: Often combined with CT, this scan shows areas of high metabolic activity where cancer cells might be growing
• MRI (magnetic resonance imaging): Uses magnets and radio waves to create detailed images, especially useful for looking at the brain and spine
• Ultrasound: Uses sound waves to create images of lymph nodes and organs

The PET/CT scan is particularly important for determining the stage of lymphoma and planning treatment.^[5]

Bone marrow biopsy: In some cases, a sample of bone marrow (the spongy tissue inside certain bones where blood cells are made) may be taken to see if lymphoma has spread there.^[2]

Treatment options

Treatment for lymphoma depends on many factors, including the type of lymphoma, its stage (how far it has spread), your age, overall health, and personal preferences. Some people with slow-growing lymphoma may not need treatment right away.^[1][2]

Active surveillance (watch and wait): For slow-growing, low-grade non-Hodgkin lymphoma with no symptoms, doctors may recommend careful monitoring without immediate treatment. You’ll have regular check-ups, and treatment will begin only if the disease starts to progress or cause symptoms.^[11][12]

Chemotherapy: This treatment uses medicines to kill cancer cells. Chemotherapy can be given through a vein (intravenously), as tablets by mouth, or in some cases as injections into the spinal fluid. It’s usually given in cycles over several months on an outpatient basis, meaning you can go home the same day.^[10][11]

For non-Hodgkin lymphoma, common chemotherapy regimens include CHOP (a combination of cyclophosphamide, doxorubicin, vincristine, and prednisone), often given with rituximab (called R-CHOP), as well as bendamustine and lenalidomide.^[5]

For Hodgkin lymphoma, treatments typically include combined chemotherapy with ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine), Stanford V, or BEACOPP regimens, often combined with radiation therapy.^[5]

Radiation therapy: This treatment uses high-energy rays to kill cancer cells. It’s most often used for early-stage lymphoma affecting only one part of the body. Treatment is usually given in short daily sessions, Monday to Friday, for up to 3 weeks. The procedure itself is painless.^[11][16]

Immunotherapy: These treatments work with your immune system to fight cancer. They include antibody treatments (like rituximab) that attach to proteins on cancer cells and help your immune system recognize and destroy them.^[10][11]

Targeted therapy: These drugs target specific features of cancer cells. They can be very effective with fewer side effects than traditional chemotherapy.^[11]

CAR T-cell therapy: This newer treatment involves collecting your own immune cells, modifying them in a laboratory to better recognize and attack cancer cells, and then returning them to your body.^[11]

Stem cell transplantation: For some patients, especially those with lymphoma that returns after initial treatment, high-dose chemotherapy followed by a stem cell or bone marrow transplant may be recommended. This procedure replaces damaged bone marrow with healthy stem cells.^[5][11]

Outlook and survival

Many people with lymphoma can be successfully treated, and some types can be cured. The outlook depends on the specific type of lymphoma, the stage at diagnosis, and how well it responds to treatment.^[2]

The five-year survival rate for all types of Hodgkin lymphoma in the United States is 85%. Treatment is highly successful for the majority of Hodgkin lymphoma patients.^[1][4]

For non-Hodgkin lymphoma, the five-year survival rate in the United States is 72%, though this varies widely depending on the specific subtype. Slow-growing (indolent) non-Hodgkin lymphomas are not currently curable, but patients can live for 20 years or more after diagnosis. Many fast-growing (aggressive) non-Hodgkin lymphomas can be cured with treatment.^[3][4][11]

Overall survival rates have been improving thanks to advances in diagnosis and treatment. The median age of diagnosis for non-Hodgkin lymphoma is 67 years.^[5]

Living with lymphoma

Coping with a lymphoma diagnosis and treatment involves both physical and emotional challenges. Each person’s experience is unique, but there are strategies that can help.

Build a strong support system: Talk to family, friends, doctors, and counselors about your fears and concerns. Consider joining a support group or peer support program to connect with others who are also coping with cancer. Writing your concerns in a journal can also help.^[18][19]

Maintain a healthy lifestyle: Eating a healthy diet rich in fruits, vegetables, proteins, and whole grains helps your body prepare for treatment and recover afterward. A balanced diet that includes 5-10 servings of fruits and vegetables daily, with at least one serving of vegetables like broccoli, cauliflower, or kale, is recommended. Regular physical activity (2.5 hours per week of moderate exercise like walking or dancing) can help control weight, improve mood, reduce fatigue, and enhance overall well-being.^[18][20][25]

Reduce your risk of infection: Because lymphoma and its treatments weaken the immune system, it’s important to wash hands frequently, avoid large crowds during cold and flu season, and ask your doctor about getting vaccinated against flu and pneumonia.^[5][25]

Manage physical changes: Hair loss and other changes in appearance caused by treatment are common. Planning ahead by purchasing a wig or head covering, and asking your healthcare team for advice on managing skin changes and other side effects, can help you feel more prepared.^[18]

Seek help for emotional challenges: Feeling sad, anxious, or depressed is not unusual when living with cancer. Watch for signs of depression such as sleeping more or less than usual, loss of interest in activities you used to enjoy, or difficulty concentrating. If these symptoms last more than two weeks, ask for a referral to a mental health professional who can help through counseling, medication, or both.^[18][19]

Stay connected: Try to maintain your normal lifestyle as much as possible. Continue your social activities, spend time with loved ones, and engage in hobbies that bring you pleasure. Don’t isolate yourself from others.^[19][23]

Follow-up care: After treatment, you’ll need regular surveillance visits to monitor for complications and relapse. Patients typically have intensive follow-up for the first two years following remission. Continue with age-appropriate health screenings as recommended by your doctor.^[5]