Hodgkin lymphoma: Full guide

What is Hodgkin lymphoma?

Hodgkin lymphoma is a cancer of lymphocytes. Lymphocytes are white blood cells that help fight infection. They live in your lymph nodes, spleen, bone marrow, and blood. Lymph nodes are small, bean-shaped glands in your neck, armpits, chest, belly, and groin.

In Hodgkin lymphoma, a small number of large, unusual cells grow in the lymph nodes. They are called Reed-Sternberg cells. These cells send out signals that pull many normal immune cells around them. That is why the nodes swell. The most common first sign is a painless lump in the neck, above the collarbone, or in the armpit. Some people have a mass in the chest that is found on an X-ray.

Some people have fevers, heavy night sweats that soak the sheets, or weight loss without trying. Doctors call these "B symptoms." Others have itching all over the body or feel very tired. Many people feel fine.

Hodgkin lymphoma is most common in teens and young adults (ages 15 to 35) and again in people over 60. Having had the Epstein-Barr virus (the virus that causes mono), having HIV, or having a close family member with Hodgkin lymphoma can raise the risk a little. Most people have none of these. The goal of treatment is cure. More than 8 out of 10 people are cured, and in early stage it is more than 9 out of 10. Because most people live a long life after treatment, care teams also work hard to lower long-term side effects.

Types

Classic Hodgkin lymphoma

About 9 out of 10 people have this type. The Reed-Sternberg cells carry a marker called CD30. It is treated with chemotherapy, sometimes with radiation, and often with a newer targeted or immune drug.

Nodular sclerosis

The most common kind of classic Hodgkin lymphoma. It often starts in the lymph nodes in the chest and neck, and is common in teens and young adults.

Mixed cellularity

A kind of classic Hodgkin lymphoma that is more common in older adults and in people with HIV. It is often linked to the Epstein-Barr virus.

Lymphocyte-rich and lymphocyte-depleted

Two less common kinds of classic Hodgkin lymphoma. Lymphocyte-rich often acts gently. Lymphocyte-depleted is rare and is more often found at a later stage. Both are treated like other classic Hodgkin lymphoma.

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL)

A rare type, about 5 out of 100 people. Its cells look and act more like a B-cell lymphoma, so it is now sometimes called nodular lymphocyte-predominant B-cell lymphoma. It usually grows slowly, is often found early, and carries the CD20 marker instead of CD30. Treatment is often lighter, and some people only need radiation or close watching.

Hodgkin lymphoma in children and teens

Young people are often treated in a children's cancer center using plans built for growing bodies. Those plans aim to cure while using less radiation.

Hodgkin lymphoma in older adults

People over 60 may have more health problems and more side effects from treatment. Plans are often changed to be gentler, for example by leaving out the drug bleomycin.

Tests you may have

Lymph node biopsy

A surgeon removes a whole lymph node, or a needle takes several thin pieces. A specialist looks at it under a microscope. A larger sample is best, because Reed-Sternberg cells are few. A small needle sample alone is often not enough.

PET-CT scan

A scan that shows active lymphoma, which lights up because it uses more sugar. It is used to find the stage, and it is often repeated after 2 or 3 cycles of treatment to see how well it is working.

Blood tests

A complete blood count, liver and kidney tests, and a test for inflammation called ESR (sed rate). The ESR helps decide your risk group in early stage.

Infection tests

Tests for HIV, hepatitis B, and hepatitis C. These matter before treatment, because some infections need care or prevention at the same time.

Heart test

An ultrasound of the heart (echocardiogram) or a similar scan. It checks how well your heart pumps before you get doxorubicin, a drug that can affect the heart.

Breathing test

Pulmonary function tests check your lungs before you get bleomycin, a drug that can harm the lungs.

Pregnancy test and fertility visit

People who could be pregnant get a pregnancy test. Anyone who may want children later should talk about saving sperm, eggs, or embryos before treatment starts.

Bone marrow biopsy

Not needed for most people, because the PET-CT scan can usually show if lymphoma is in the bone marrow. It may be done if blood counts are low for no clear reason.

Stages and risk groups

The stage tells how far the lymphoma has spread. Hodgkin lymphoma often spreads in an orderly way, from one group of lymph nodes to the next. A high stage does not mean it cannot be cured. Most people with stage 3 or 4 Hodgkin lymphoma are cured.

Your stage may also have a letter. "A" means no B symptoms. "B" means fever, drenching night sweats, or weight loss. "E" means it has grown into an organ next to a lymph node. "Bulky" means one very large mass, often in the chest.

Early-stage Hodgkin lymphoma is split into favorable and unfavorable groups. Unfavorable features include a large mass in the chest, a high ESR, lymphoma in many lymph node areas, or spread outside the lymph nodes. People in the unfavorable group usually get a little more treatment.

Stage 1

Lymphoma is in one lymph node area, or in one organ outside the lymph nodes.

Stage 2

Lymphoma is in two or more lymph node areas on the same side of the diaphragm. The diaphragm is the muscle under your lungs.

Stage 3

Lymphoma is in lymph nodes both above and below the diaphragm, or in the spleen.

Stage 4

Lymphoma has spread more widely into organs such as the lungs, liver, bone, or bone marrow.

Early favorable

Stage 1 or 2 with none of the unfavorable features. This group usually needs the least treatment.

Early unfavorable

Stage 1 or 2 with one or more unfavorable features. Treatment is a bit longer or may include radiation.

Advanced stage

Stage 3 or 4, and in some plans stage 2 with B symptoms or a bulky mass. A risk score called the IPS uses age, sex, blood counts, and albumin to help predict how the lymphoma may behave.

Lab tests and PET results

Lab tests on your biopsy confirm that you have Hodgkin lymphoma and show which type. These tests use special stains that look for markers on the cells.

One of the most important results comes later, from the PET-CT scan done partway through treatment. If the scan is clear early, many people can have less treatment. If it still shows active lymphoma, the plan may be made stronger. This is called PET-guided or PET-adapted treatment.

CD30

A marker found on the Reed-Sternberg cells of classic Hodgkin lymphoma. The drug brentuximab vedotin attaches to CD30 and carries a cancer drug into the cell.

CD15

Another marker often found on classic Hodgkin lymphoma cells. It helps the lab confirm the diagnosis.

CD20

A marker found on the cells of nodular lymphocyte-predominant Hodgkin lymphoma. It means the antibody drug rituximab can be part of treatment for that type.

PD-L1 and the 9p24.1 gene change

Classic Hodgkin lymphoma cells often have extra copies of genes that help them hide from the immune system. This is why immune drugs called checkpoint inhibitors, such as nivolumab and pembrolizumab, work so well in this disease. You do not need a test for this before getting them.

Epstein-Barr virus (EBV)

The lab may check if the virus is inside the lymphoma cells. It is found more often in older adults and in people with HIV. It does not change the main treatment for most people.

Deauville score

A 1 to 5 score used to read PET scans. A score of 1, 2, or 3 usually means the scan is clear (PET-negative). A score of 4 or 5 means there may still be active lymphoma (PET-positive).

Interim PET

A PET-CT scan done after the first 2 or 3 cycles. It helps decide whether to drop a drug, skip radiation, or make treatment stronger.

End-of-treatment PET

A scan at the end of treatment that shows if you are in complete remission. It also helps decide if radiation to one area is needed.

Main treatments

ABVD and AVD chemotherapy

ABVD stands for 4 drugs: doxorubicin (Adriamycin), bleomycin, vinblastine, and dacarbazine. It is given by vein every 2 weeks. It has been the backbone of Hodgkin treatment for decades. If an early PET scan is clear, bleomycin is often dropped (AVD) to protect the lungs.

Nivolumab plus AVD (N-AVD)

Nivolumab is an immune drug called a checkpoint inhibitor. It takes the brakes off your immune system. Given with AVD, it is now a standard first treatment in the US for teens and adults with stage 3 or 4 classic Hodgkin lymphoma. It is also being studied in earlier stages.

Brentuximab vedotin plus AVD (BV-AVD)

Brentuximab vedotin is an antibody-drug conjugate. It attaches to CD30 and carries a cancer drug straight into the lymphoma cell. Given with AVD, it is another first treatment for advanced stage. It can cause nerve damage, and people need a shot to boost white blood cells.

BEACOPP variants

Escalated BEACOPP is a stronger 7-drug plan used mainly in Europe for younger adults with advanced stage. A newer version called BrECADD adds brentuximab vedotin and drops some drugs. It cures more people than escalated BEACOPP, with fewer side effects. These plans are given for 4 or 6 cycles based on the PET scan. They carry a higher risk of infertility.

Treatment for early stage

Most people get 2 to 4 cycles of ABVD or AVD. Some also get a short course of radiation to the area where the lymphoma started. A clear PET scan after chemotherapy may allow some people to skip radiation. Your care team will weigh a small lower chance of relapse against the long-term risks of radiation.

Radiation therapy

High-energy beams aimed only at the lymph nodes that had lymphoma. Today's radiation uses lower doses and smaller areas than in the past, which lowers long-term risks. It is given on weekdays for 2 to 4 weeks.

Treatment for nodular lymphocyte-predominant type

Early stage is often treated with radiation alone. Some people whose only lymph node was fully removed may be watched. Advanced stage may be treated with rituximab alone or with rituximab plus chemotherapy, such as R-CHOP.

Treatment if Hodgkin lymphoma comes back

Options include a different chemotherapy plan, often with brentuximab vedotin or a checkpoint inhibitor such as pembrolizumab or nivolumab. If the lymphoma responds, many people then have high-dose chemotherapy and an autologous stem cell transplant (using their own stem cells). Some get brentuximab vedotin afterward to lower the chance of another relapse. Many people are still cured at this point.

Checkpoint inhibitors and donor transplant for later relapse

Pembrolizumab or nivolumab alone can control Hodgkin lymphoma for a long time, even after a transplant. Some people have an allogeneic stem cell transplant, which uses cells from a donor. Clinical trials are another good option.

Common side effects and what helps

Infections and low white blood cells

Chemotherapy lowers the white blood cells that fight germs. Wash your hands often and stay away from sick people. Call right away for a fever of 100.4 F (38 C) or higher. People getting BV-AVD, and some others, get shots of a growth factor to raise white cell counts.

Nausea and vomiting

Dacarbazine can cause nausea on the day of treatment. Take your anti-nausea medicine on schedule, before you feel sick. Small meals and plenty of fluids help.

Tiredness

Fatigue is common and often builds over the months of treatment. Short daily walks, rest breaks, and help from family and friends can make a difference.

Lung damage from bleomycin

Bleomycin can rarely inflame and scar the lungs. Report a new cough or shortness of breath. Do not smoke. Tell any surgeon or anesthesia doctor that you had bleomycin, because high levels of oxygen can harm the lungs later.

Numbness or tingling (neuropathy)

Vinblastine and especially brentuximab vedotin can cause numbness, tingling, or pain in the hands and feet, or trouble with buttons. Tell your care team early, because changing the dose can keep it from getting worse. It often gets better slowly after treatment.

Immune side effects from nivolumab or pembrolizumab

These drugs can make the immune system attack healthy organs. This can cause thyroid problems, rash, diarrhea, liver problems, or a cough. Most are mild and treatable, but report any new symptom, even months later. Thyroid problems may need lifelong thyroid pills.

Hair loss and mouth sores

Hair often thins or falls out with ABVD and AVD. It grows back after treatment. Rinse your mouth with salt and baking soda water and use a soft toothbrush for mouth sores.

Skin and throat changes from radiation

The treated skin may get red or dry. Radiation to the neck or chest can cause a sore throat or trouble swallowing for a few weeks. Your radiation team can suggest creams and soothing rinses.

Changes to periods and fertility

Periods may stop during treatment. With ABVD and AVD, most people's fertility comes back. With BEACOPP-type plans, the risk of lasting infertility and early menopause is higher. Use birth control during treatment, because the drugs can harm a baby.

Living with and after Hodgkin lymphoma

After treatment, you will have regular check-ups, often every few months at first. Most people do not need routine scans once they are in remission and feel well. Tell your care team about new lumps, fevers, night sweats, itching, or weight loss. Most relapses happen in the first 2 to 3 years.

Because most people are cured young, long-term health matters a lot. Doxorubicin and chest radiation can raise the risk of heart disease years later. Radiation to the neck can cause an underactive thyroid. Bleomycin can affect the lungs. Treatment can also slightly raise the risk of other cancers later in life, such as breast, lung, or thyroid cancer.

Ask for a written survivorship plan. It should list the treatment you had and the follow-up you need. This may include yearly thyroid blood tests, heart checks, and blood pressure and cholesterol checks. If you had radiation to the chest before age 30, breast screening with MRI and mammograms often starts early, at age 25 or 8 years after radiation, whichever comes later.

If you want children, talk about fertility before treatment starts. Sperm banking is quick and simple. Egg or embryo freezing takes about 2 weeks and can often be done without delaying treatment much. Many people have healthy children after Hodgkin lymphoma. Ask how long to wait after treatment before trying to get pregnant. Not smoking is one of the best things you can do, because smoking adds to the lung and heart risks of treatment. Staying active, eating well, and keeping up with vaccines also help. It is normal to feel worried or low after treatment ends. Support groups, counselors, and young adult cancer programs can help.