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Cancer Medicine :: Hodgkin Disease Treatment

Hodgkin Disease

General treatment information

After Hodgkin disease is staged, the cancer care team will discuss treatment options with you. Treatment for Hodgkin disease is based largely on the stage of the disease. But other factors, including a person’s age and general health, and the type and location of the disease, may also affect treatment options.

For almost all patients with Hodgkin disease, cure is the main goal. But treatment can have side effects that often don’t show up for many years. Because of this, doctors try to choose a treatment plan with the lowest risk of potential side effects.

Several types of treatment can be used for Hodgkin disease:

Chemotherapy for Hodgkin disease

Chemotherapy (chemo) is the use of drugs to kill cancer cells. The drugs can be taken as pills or injected into a vein under the skin. Chemotherapy is systemic therapy, which means the drugs enter the bloodstream and travel throughout the body to reach and destroy cancer cells wherever they may be.

Doctors give chemo in cycles, in which a period of treatment is followed by a rest period to give the body time to recover. Each cycle generally lasts for several weeks. Most chemo treatments are given on an outpatient basis (in the doctor’s office, clinic, or hospital outpatient department), but some may require a hospital stay.

The chemo regimens for Hodgkin disease combine several drugs because different drugs kill cancer cells in different ways. The combinations used to treat Hodgkin disease are often referred to by abbreviations that are easier to remember than the drugs’ full names. The most common regimen in the United States is a 4-drug combination called ABVD, which consists of: Adriamycin® (doxorubicin), Bleomycin, Vinblastine, Dacarbazine (DTIC)

Other common regimens include:

Stanford V:  Doxorubicin (Adriamycin), Mechlorethamine (nitrogen mustard), Vincristine, Vinblastine, Bleomycin, Etoposide, Prednisone

BEACOPP:Bleomycin,Etoposide(VP-16),Adriamycin(doxorubicin),Cyclophosphamide Cytoxan®),Oncovin® (vincristine),Procarbazine,Prednisone

Radiation is given after chemo in the Stanford V regimen, and it is sometimes given after the ABVD or BEACOPP regimens as well.

Other chemotherapy combinations may also be used for Hodgkin disease. Most use the same drugs as listed above, but they may include different combinations and be given on different schedules.

Possible side effects

Chemo drugs attack cells that are dividing quickly, which is why they work against most types of lymphoma cells. But other cells in the body, such as those in the bone marrow, the lining of the mouth and intestines, and the hair follicles, also divide quickly. These cells are also likely to be affected by chemotherapy, which can lead to side effects.

The side effects depend on the type and dose of drugs given and the length of time they are taken. They can include:

Hair loss, Mouth sores, Loss of appetite, Nausea and vomiting, Diarrhea, Increased chance of infections (from having too few white blood cells), Easy bruising or bleeding (from having too few blood platelets), Fatigue (from having too few red blood cells).

These side effects are usually short-term and go away after treatment is finished. If serious side effects occur, the chemotherapy may have to be delayed or the doses reduced.

There are often ways to lessen these side effects. For example, drugs are usually given to help prevent nausea and vomiting.

Drugs known as growth factors, such as G-CSF (Neupogen®) or GM-CSF (Leukine®), are sometimes given to help the body make more white blood cells and thus reduce the chance of infection. Antibiotics may also be given at the earliest sign of an infection, such as a fever.

If your white blood cell counts are very low during treatment, you can help lower the risk of infection by carefully limiting your exposure to germs. During this time, your doctor may advise you to:

Wash your hands often.

Avoid fresh, uncooked fruits and vegetables and other foods that might carry germs.

Avoid fresh flowers and plants because they may carry mold.

Make sure other people wash their hands before they come in contact with you.

Avoid large crowds and people who are sick (wearing a surgical mask offers some protection in these situations).

Your platelet counts are very low, you may be given drugs or platelet transfusions to help protect against bleeding. Fatigue caused by anemia (very low red blood cell counts) can be treated with drugs or with red blood cell transfusions.

Long-term side effects: Some chemo drugs can have long-term side effects that occur months or years after treatment has ended.

For example, doxorubicin can cause heart damage, so your doctor may order a test to check your heart function before and during treatment with this drug.

Bleomycin can cause lung damage, so some doctors order tests of lung function (called pulmonary function tests) before starting patients on this drug.

Some chemo drugs can increase the risk of getting a second type of cancer later in life (such as leukemia), especially in patients who have also received radiation therapy.

In children and young adults, some chemo drugs can also affect body growth and fertility (ability to have children) later on.

Before starting chemotherapy, ask your doctor to explain the possible side effects and your chances of having them.

Radiation therapy for Hodgkin disease

Radiation therapy uses high-energy rays (or particles) to destroy cancer cells.

To treat Hodgkin disease, a carefully focused beam of radiation is delivered from a machine outside the body. This is known as external beam radiation. Most often, radiation treatments are given 5 days a week for several weeks. Before the treatments start, the radiation team takes careful measurements to determine the dose needed and the correct angles for aiming the radiation beams. The treatment is much like getting an x-ray, but the radiation is more intense. Each treatment lasts only a few minutes, although the setup time – getting you or your child into place – usually takes longer. Radiation itself is painless, but some younger children may need to be sedated to make sure they don’t move during the treatment.

Radiation therapy is most useful when Hodgkin disease is only in one part of the body. For classic Hodgkin disease, radiation is often given after chemotherapy, especially when there is a large or bulky tumor mass (usually in the chest). The chemotherapy or radiation alone would probably not cure the disease, but both treatments together are usually effective in getting rid of it. Radiation therapy may also be used by itself to treat some cases of nodular lymphocyte predominant Hodgkin disease.

Radiation therapy is often very good at killing Hodgkin disease cells. Decades ago, this was the best treatment available for Hodgkin disease, but over the years doctors realized it could lead to long-term side effects. As it became clear that chemotherapy was also effective, doctors began to use less radiation. Today, if radiation therapy is used, only the involved areas are treated with radiation to try to limit side effects.

Involved field radiation

This is the preferred form of radiation therapy when it is used to treat Hodgkin disease. Only the lymph node areas that contain Hodgkin disease are treated. Chemotherapy is typically given first, and then radiation is given to areas that were initially involved.

Extended field radiation

In the past, radiation was given to the major lymph node areas that contained Hodgkin disease, as well as the surrounding “normal” lymph node areas, just in case the Hodgkin disease had spread, even though the doctors could not actually detect it in these areas. This is called extended field radiation.

If the Hodgkin disease was in the upper body, radiation was given to the mantle field, which included lymph node areas in the neck, chest, and under the arms. Sometimes this was extended to also include lymph nodes in the upper abdomen.

Inverted Y field radiation therapy included the lymph nodes in the upper abdomen, the spleen, and the lymph nodes in the pelvis).

When inverted Y field radiation was given together with mantle field radiation, the combination was called total nodal irradiation.

Because nearly all patients with Hodgkin disease are now treated with chemotherapy, extended field radiation is seldom used any more.

Total body irradiation

People who are getting a stem cell transplant may get radiation to the whole body along with high-dose chemotherapy, to try to kill lymphoma cells throughout the body.

Possible side effects

The side effects of radiation therapy depend on where the radiation is aimed. Some people have skin changes similar to sunburn, which slowly fades away. Other possible short-term side effects include fatigue, dry mouth, nausea, or diarrhea. Radiation given to several areas, especially after chemotherapy, can lower blood cell counts.

Long-term side effects: Radiation therapy can also have long-term side effects. The most serious of these is the development of another cancer in the part of the body that was exposed to radiation.

Radiation to the chest or neck can damage the thyroid gland, which can affect its ability to make thyroid hormone. This can lead to fatigue and weight gain. Treatment with thyroid hormone pills can help with this. Radiation to the chest also increases the risk of heart disease (such as heart attacks) and lung problems, while radiation to the neck may increase the risk of stroke many years later.

In children, radiation that reaches the bones may slow their growth. Depending on where the radiation is given, this could result in deformities or a lack of growth to full height. Radiation to the lower part of the body in children and young adults might also affect fertility later in life.

To reduce the risk of side effects, doctors carefully calculate the exact dose of radiation needed and aim the radiation beam as accurately as they can. Shields may also be placed over nearby parts of the body to protect them from the radiation. In girls and young women, the ovaries may be moved out of the way with minor surgery before radiation is given to help preserve fertility.

Monoclonal antibodies for Hodgkin disease

Antibodies are proteins made by your immune system to help fight infections. Man-made versions, called monoclonal antibodies, can be designed to attack a specific target, such as a substance on the surface of lymphocytes (the cells in which lymphomas start).

Some monoclonal antibodies are now being used to treat Hodgkin disease.

Brentuximab vedotin (Adcetris™): This drug is an anti-CD30 antibody attached to a chemotherapy drug. Hodgkin disease cells usually have the CD30 molecule on their surface. The antibody acts like a homing signal, bringing the chemo drug to the lymphoma cells, where it enters the cells and causes them to die when they try to divide into new cells.

This drug has been shown to help many people whose Hodgkin disease has come back after other treatments, including a stem cell transplant (see next section), as well as people who can’t have a stem cell transplant. It is also being studied to see if it can be given with chemotherapy and if can be helpful earlier in the course of the disease.

Brentuximab is given as an infusion into a vein (IV) every 3 weeks. Common side effects include nerve damage (neuropathy), low blood counts, fatigue, fever, nausea and vomiting, infections, diarrhea, and cough. Rarely, more severe side effects occur during infusions, such as trouble breathing and low blood pressure.

Rituximab (Rituxan®): This antibody attaches to a substance called CD20 found on some types of lymphoma cells, which seems to cause the lymphoma cell to die. Rituximab may be used to treat nodular lymphocyte predominant Hodgkin disease (NLPHD), often with chemotherapy and/or radiation therapy.

Rituximab is given as an IV infusion in the doctor’s office or clinic. When it is used by itself to treat lymphoma, it is usually given once a week for 4 weeks, which may then be repeated several months later. When it is combined with chemotherapy, it is most often given on the first day of each chemo cycle.

Common side effects are usually mild but may include chills, fever, nausea, rashes, fatigue, and headaches. Rarely, more severe side effects occur during infusions, such as trouble breathing and low blood pressure. Even if these symptoms occur during the first rituximab infusion, it is very unusual for them to recur with later doses. Rituximab can cause dormant (inactive) hepatitis B infections to become active again, sometimes leading to severe liver problems or even death. Your doctor may check your blood for signs of hepatitis before starting this drug. This drug may also increase the risk of certain infections for several months after the drug is stopped.

High-dose chemotherapy and stem cell transplant for Hodgkin disease

Stem cell transplants (SCTs) are sometimes used for Hodgkin disease that is hard to treat, such as those that don’t go away completely after chemotherapy and/or radiation, or those that come back after treatment.

The doses of chemotherapy drugs normally are limited by the side effects these drugs cause. Higher doses can’t be used, even if they might kill more cancer cells, because they would severely damage the bone marrow, where new blood cells are made.

A stem cell transplant lets doctors use higher doses of chemotherapy (sometimes along with radiation therapy). After getting high-dose treatment, the patient receives a transplant of blood-forming stem cells to restore the bone marrow.

Blood-forming stem cells used for a transplant come either from the blood (for a peripheral blood stem cell transplant, or PBSCT) or from the bone marrow (for a bone marrow transplant, or BMT). Peripheral blood stem cells are obtained from a procedure similar to a blood donation, while bone marrow donation is usually done in an operating room with the marrow donor under general anesthesia (in a deep sleep). Bone marrow transplants were more common in the past, but they have largely been replaced by PBSCTs.

Types of transplants

There are 2 main types of stem cell transplants. They differ with regard to the source of the blood-forming stem cells.

Autologous stem cell transplant

In this type of transplant, a patient’s own blood stem cells are removed from his or her bone marrow or peripheral blood. They are collected several times in the weeks before treatment. The cells are frozen and stored while the person gets treatment (high-dose chemotherapy and/or radiation) and then are given back to the patient by infusion into the patient’s blood. This is the more common type of transplant for Hodgkin disease.

Allogeneic stem cell transplant

In this type of transplant, the blood stem cells come from someone else. The donor’s tissue type (also known as the HLA type) needs to match the patient’s tissue type as closely as possible to help prevent the risk of major problems with the transplant.

Usually the donor is a brother or sister if they have the same tissue type as the patient. If there are no siblings who are a good match, the cells may come from an HLA-matched, unrelated donor — a stranger who has volunteered to donate their cells.

Sometimes umbilical cord blood stem cells are used. These cells come from blood drained from the umbilical cord and placenta after a baby is born, which is rich in blood stem cells. These are more often a source of blood stem cells for transplants in children.

Regardless of the source, the stem cells are then frozen and stored until they are needed for the transplant.

Allogeneic transplants may be more likely to rid the body of lymphoma cells, but they are also more likely to cause serious complications that could be life-threatening. In treating Hodgkin disease, an allogeneic transplant is generally used only if an autologous transplant has already been tried without success.

Non-myeloablative transplant: This special type of allogeneic transplant may be an option for some patients who couldn’t tolerate a regular allogeneic transplant because it would be too toxic. This type of transplant uses less chemo and radiation, so it is also known as a mini-transplant or reduced-intensity transplant.

The lower doses of chemotherapy and radiation do not completely destroy the cells in the bone marrow. After treatment, the patient gets the allogeneic (donor) stem cells. These cells establish a new immune system in the body, which sees the lymphoma cells as foreign and attacks them (called the graft-versus-lymphoma effect).

Even though it uses small doses of certain chemotherapy drugs and low doses of total body radiation, this type of transplant can still sometimes work and have less serious side effects. In fact, some patients can receive a non-myeloablative transplant as an outpatient.

Doctors aren’t yet sure exactly how effective these types of transplants are for patients with Hodgkin disease, but studies are now being done to find out.

The transplant procedure

The patient may be admitted to the stem cell transplant unit of the hospital or receive treatment as an outpatient depending on a number of factors.

If they are going to be treated in the hospital, the person is usually admitted to the hospital the day before the high-dose chemo is to begin. He or she will usually stay in the hospital until the stem cells have started to make new blood cells again, which often takes several weeks.

If the transplant is done as an outpatient procedure, patients and families must be able to spot complications requiring their doctor’s attention. Unless they live close to the transplant center, they will be asked to stay in a nearby hotel.

Treatment starts with high-dose chemo and may include high-dose whole body radiation. The chemo and radiation treatments are meant to destroy any remaining cancer cells. They also kill the normal cells of the bone marrow and the immune system. Once treatment is complete, the new stem cells (autologous or allogeneic) are given through a vein, just like a blood transfusion. The stem cells then migrate to the bone marrow.

In an allogeneic SCT, the person getting the transplant may be given drugs to keep the new immune system from attacking the body. This is known as graft-versus-host disease or GVHD. For the next several weeks the patient is likely to have very low blood cell counts, so they are given as much supportive therapy as needed. This may include antibiotics, red blood cell or platelet transfusions, other medicines, and help with nutrition.

Usually within a couple of weeks after the stem cells have been infused, they begin making new white blood cells. This is later followed by the new platelet production and new red blood cell production. Because of the high risk of serious infections right after treatment, patients remain protected in isolation (where exposure to germs is kept to a minimum) until a measure of their white blood cells, the absolute neutrophil count (ANC), rises above 500. They can usually leave the hospital when their ANC nears 1,000.

Patients then typically make regular visits to the outpatient transplant clinic for about 6 months, after which their care is continued by their regular doctors. At this point, they may only come back to the clinic for their regular exams or if they have symptoms that should be checked by their doctor.

Practical points

A stem cell transplant is a complex treatment that can cause life-threatening side effects. If the doctors think a patient may benefit from a transplant, it should be done at a hospital where the staff has experience with the procedure and with managing the recovery phase. Some stem cell transplant programs may not have experience in certain types of transplants, especially transplants from unrelated donors.

SCTs often require a long hospital stay and can be very expensive (costing well over $100,000). Because some insurance companies may view it as an experimental treatment, they may not pay for it. Even if the transplant is covered by your insurance, your co-pays or other costs could easily amount to tens of thousands of dollars. Find out what your insurer will cover before deciding on a transplant so you will have an idea of what you might have to pay.

Possible side effects

Early or short-term effects: The early complications and side effects are basically the same as those caused by any other type of high-dose chemotherapy . They are caused by damage to the bone marrow and other quickly growing tissues of the body and can include:

Low blood cell counts (with fatigue and increased risks of infection and bleeding)

Nausea and vomiting

Diarrhea

Loss of appetite

Mouth sores

Hair loss

One of the most common and serious short-term effects is the increased risk for infection. Antibiotics are often given to try to prevent this from happening. Other side effects, like low red blood cell and platelet counts, may require blood product transfusions or other treatments.

Long-term side effects: Some complications and side effects can last for a long time or occur many years after the transplant. These can include:

Graft-versus-host disease, which occurs only in allogeneic transplants (see next paragraph)

Menstrual changes, early menopause, and loss of fertility in female patients (caused by damage to the ovaries)

Loss of fertility in male patients

Damage to the thyroid gland, causing problems with metabolism

Cataracts (damage to the lens of the eye that can affect vision)

Damage to the lungs, causing shortness of breath

Bone damage called aseptic necrosis (if damage is severe, the patient may need to have part of the affected bone and the joint replaced)

Development of leukemia or another cancer years later

Graft-versus-host disease (GVHD): This is one of the most serious complications of allogeneic (donor) stem cell transplants. It occurs because the immune system of the patient is replaced by the donor’s immune system. The donor immune system then may “see” the patient’s own body tissues as foreign and may react against them.

Symptoms can include severe skin rashes, itching, mouth sores (which can affect eating), nausea, and severe diarrhea. Liver damage may cause yellowing of the skin and eyes (jaundice). The lungs may also be damaged. The patient may also tire easily and have muscle aches.

GVHD is either acute or chronic, based on how soon after the transplant it begins. Sometimes GVHD can become disabling, and if it is severe enough, it can be life-threatening. Usually, immune-suppressing drugs can be used to help control GVHD, although they can have their own side effects.

On the positive side, the graft-versus-host disease also leads to graft-versus-lymphoma activity. Lymphoma cells remaining after the chemotherapy and radiation therapy are often killed by donor immune cells since the lymphoma cells are seen as foreign by the donor’s immune system as well. Mild graft-versus-host disease can be a good thing.

The 2 main methods of treating Hodgkin disease are chemotherapy (the use of cancer-killing drugs) and radiation therapy (the use of high-energy rays or particles). Depending on the situation, one or both of these treatments might be used.

Monoclonal antibodies and high-dose chemotherapy with stem cell transplants may be used for certain patients, especially if other treatments haven’t worked. Except for biopsy and staging, surgery is rarely used to treat Hodgkin disease.

Based on your treatment options, you may have different types of doctors on your treatment team. These doctors may include:

A hematologist: a doctor who treats disorders of the blood, including lymphomas.

A medical oncologist: a doctor who treats cancer with medicines.

A radiation oncologist: a doctor who treats cancer with radiation therapy.

Many other specialists may be involved in your care as well, including nurse practitioners, nurses, nutrition specialists, social workers, and other health professionals.

It is important to discuss all of your treatment options as well as their possible side effects with your doctors to help make the decision that best fits your needs. In choosing a treatment plan, consider your health and the type and stage of the Hodgkin disease. Be sure that you understand all the risks and side effects of the various treatments before making a decision.

If time permits, it is often a good idea to seek a second opinion. Getting a second opinion can give you more information and help you feel confident about the treatment plan that you choose. Your doctor should be willing to help you find another cancer doctor who can give you a second opinion.

The next few sections describe the types of treatments used for Hodgkin disease. This is followed by a discussion of the typical treatment options based on the stage of the disease (and other prognostic factors when these are important). Finally, treatment of Hodgkin disease in special circumstances, such as in children or during pregnancy, is discussed.

What happens after treatment for Hodgkin disease?

For many people with Hodgkin disease, treatment may remove or destroy the cancer. Completing treatment can be both stressful and exciting. You may be relieved to finish treatment, but find it hard not to worry about the lymphoma growing or coming back. (When cancer comes back after treatment, it is called recurrence.) This is a very common concern in people who have had cancer.

It may take a while before your fears lessen. But it might help to know that many cancer survivors have learned to live with this uncertainty and are leading full lives.

For some people, the lymphoma may never go away completely. These people may get regular treatments with chemotherapy, radiation therapy, or other therapies to help keep the lymphoma in check for as long as possible. Learning to live with lymphoma as more of a chronic disease can be difficult and very stressful. It has its own type of uncertainty.

Follow-up care

If you (or your child) have completed treatment, your doctors will still want to watch you closely. It is very important to keep all follow-up appointments. You or your child will need follow-up care for many years after treatment for Hodgkin disease.

During these visits, the doctor will ask about symptoms, do physical exams, and may order blood tests or imaging tests such as CT scans or chest x-rays. Doctor visits are usually recommended every few months for the first several years after treatment. Gradually, the length of time between visits can be increased, but even after 5 years they should be done at least yearly.

Follow-up is needed to check for cancer recurrence or spread, as well as possible side effects of certain treatments. This is the time for you to ask your health care team any questions you need answered and to discuss any concerns you might have.

If Hodgkin disease does come back at some point, further treatment will depend on what treatments you’ve had before, how long it’s been since treatment, and your health.

Watching for long-term side effects

Each type of treatment for Hodgkin disease has side effects that could last for months or longer. Some side effects, like loss of fertility, may be permanent. Because so many people now live for a long time after their treatment, watching for these serious side effects is very important.

Second cancers: One of the most serious side effects of Hodgkin disease treatment is developing a second cancer later on. For example, acute myelogenous leukemia (AML) can develop in a small portion of patients after receiving certain types of treatment. This usually occurs in the first few years after treatment and is seen more often in older people.

This might be less likely with current chemo drug combinations like ABVD than with some combinations used more often in the past, such as the MOPP regimen, but there is still a small risk.

Radiation may also add to this risk. Although radiation alone does not increase the risk for leukemia much, it can raise the risk of other forms of cancer in the part of the body that received the radiation.

Women who receive chest radiation before they are 30 years old have a much higher risk of breast cancer. They should be especially careful about following American Cancer Society recommendations for early detection of breast cancer and should talk to their doctor about starting screening at an early age.

Both men and women receiving chest radiation have a higher chance of developing lung cancer, mesothelioma (a cancer of the lining of the lungs), and thyroid cancer. The risk of lung cancer is much higher in smokers, so not smoking is especially important among survivors of Hodgkin disease. Follow-up physical exams, blood tests for thyroid problems, and spiral CT scans or x-rays of the chest as suggested by your doctor may be helpful.

Cancers of muscle or bone, called sarcomas, can also develop in areas that get radiation. Likewise, digestive tract cancers such as colon cancer are also more likely.

Another type of cancer, non-Hodgkin lymphoma, develops in a small number of patients with Hodgkin disease. It is thought that this risk is due mostly to the disease itself and not the treatment.

Fertility issues: A possible long-term effect of chemotherapy and radiation therapy, especially in younger patients, is reduced or lost fertility. For example, some chemotherapy drugs might affect a male’s ability to make sperm, which might be temporary or permanent. If the patient is old enough and is going to get drugs that can affect fertility, sperm banking should be considered before chemotherapy is started.

Likewise, women may stop ovulating and menstruating with chemotherapy. This may or may not return to normal. Radiation to the lower abdomen can cause infertility unless the ovaries are surgically moved outside the radiation field beforehand. Moving the ovaries does not affect cure rates because Hodgkin disease almost never spreads to the ovaries.

Infections: For unknown reasons, the immune system of people with Hodgkin disease does not work properly. Treatments such as radiation, chemotherapy, and surgery to remove the spleen (splenectomy) can add to this problem. Splenectomy was once common but is now rare for people with Hodgkin disease. Patients who have their spleen removed should be immunized against certain bacteria.

All people who have had Hodgkin disease should keep up with their flu shots. Keeping up with vaccinations and careful, prompt treatment of infections are very important.

Thyroid problems: Radiation therapy to the chest or neck to treat Hodgkin disease might affect the thyroid gland, causing it to make less thyroid hormone. People with this condition, known as hypothyroidism, may need to take thyroid medicine. People who got radiation to the neck or upper chest should have their thyroid function checked with blood tests at least yearly.

Heart disease and strokes: People who have had radiation to the chest have a higher risk of heart disease and heart attacks. This has become less of a problem with more modern radiation techniques, but it is important to not smoke and maintain a healthy diet to help avoid this problem. Some chemotherapy drugs such as doxorubicin (Adriamycin) and mitoxantrone can also cause heart damage. Your doctor may advise you to have tests to check your heart function several years after your treatment.

Radiation to the neck increases the chance of stroke because it can damage the blood vessels in the neck that supply the brain. Smoking and high blood pressure also increase the risk of stroke. Once again it is important to avoid smoking. It is also important to have regular check-ups with your doctor and have any high blood pressure treated.

Lung damage: The chemotherapy drug bleomycin can damage the lungs, as can radiation therapy to the chest. This can lead to problems such as shortness of breath, which might not show up until years after treatment. Smoking can also seriously damage the lungs, so it is important that people who have had these treatments do not smoke.

Special concerns in childhood Hodgkin disease survivors

Just as the treatment of childhood Hodgkin disease requires a very specialized approach, so does follow-up and monitoring for late effects of treatment. Careful follow-up after treatment is very important.

Along with physical side effects (including those listed above), survivors of childhood cancer may have emotional or psychological issues that need to be addressed. They also may have some problems with normal functioning and school work. These can often be addressed with support and encouragement. Doctors and other members of the health care team can also often recommend special support programs and services to help children after cancer treatment.

To help increase awareness of late effects and improve follow-up care of childhood cancer survivors throughout their lives, the Children’s Oncology Group (COG) has developed long-term follow-up guidelines for survivors of childhood cancers. These guidelines can help you know what to watch for, what types of health screening should be done, and how late effects may be treated.

It is very important to discuss possible long-term complications with your child’s health care team, and to make sure there is a plan in place to watch for these problems and treat them, if needed. To learn more, ask your child’s doctors about the COG survivor guidelines.

If treatment for Hodgkin disease is no longer working

If Hodgkin disease keeps growing or comes back after one kind of treatment, it is often possible to try other treatment plans that might still cure it, or at least shrink the tumors enough to help you live longer and feel better.

But when a person has tried many different treatments and the lymphoma is no longer getting better, even newer treatments may no longer be effective. If this happens, it’s important to weigh the possible limited benefits of trying a new treatment against the possible downsides, including treatment side effects. Everyone has their own way of looking at this.

This is likely to be the hardest part of your battle with cancer — when you have been through many treatments and nothing’s working anymore. Your doctor might offer you new options, but at some point you may need to consider that treatment is not likely to improve your health or change your outcome or survival.

If you want to continue to get treatment for as long as you can, you need to think about the odds of treatment having any benefit and how this compares to the possible risks and side effects. Your doctor can give you an estimate how likely it is the cancer will respond to treatment you are considering. For instance, the doctor may say that more treatment might have about a 1 in 100 chance of working. Some people are still tempted to try this. But it is important to think about and understand your reasons for choosing this plan.

No matter what you decide to do, it is important that you feel as good as you can. Make sure you are asking for and getting treatment for any symptoms you might have, such as nausea or pain. This type of treatment is called palliative care.

Palliative care helps relieve symptoms, but is not expected to cure the disease. It can be given along with cancer treatment, or can even be cancer treatment. The difference is its purpose — the main goal of palliative care is to improve the quality of your life, or help you feel as good as you can for as long as you can. Sometimes this means using drugs to help with symptoms like pain or nausea. Sometimes, though, the treatments used to control your symptoms are the same as those used to treat cancer. For instance, radiation might be used to help relieve pain caused by a large tumor. Or chemo might be used to help shrink a tumor and keep it from blocking the bowels. But this is not the same as treatment to try to cure the cancer.

At some point, you may benefit from hospice care. This is special care that treats the person rather than the disease; it focuses on quality rather than length of life. Most of the time, it is given at home. Your cancer may be causing problems that need to be managed, and hospice focuses on your comfort. You should know that while getting hospice care often means the end of treatments such as chemo and radiation, it doesn’t mean you can’t have treatment for the problems caused by your cancer or other health conditions. In hospice the focus of your care is on living life as fully as possible and feeling as well as you can at this difficult time. You can learn more about hospice in our document called Hospice Care.

Staying hopeful is important, too. Your hope for a cure may not be as bright, but there is still hope for good times with family and friends — times that are filled with happiness and meaning. Pausing at this time in your cancer treatment gives you a chance to refocus on the most important things in your life. Now is the time to do some things you’ve always wanted to do and to stop doing the things you no longer want to do. Though the cancer may be beyond your control, there are still choices you can make.

What’s new in Hodgkin disease research and treatment?

Important research into Hodgkin disease is being done right now in many university hospitals, medical centers, and other institutions around the world. Scientists are getting closer to finding out what causes the disease and how to improve treatment.

Imaging tests: In recent years, PET scans and combined PET/CT scans have been found to be very helpful in determining the extent of Hodgkin disease in the body and in assessing how well treatment is working. PET scans are now commonly used early in the course of treatment to help doctors decide how much treatment needs to be given.

Tailoring treatment: In general, cure rates for Hodgkin disease are high, but long-term side effects of treatment are an important issue. A very active area of research is directed at learning which patients can be treated with gentler therapy and which patients need stronger treatment.

A related area of research is finding less-toxic treatments that do not have serious long-term side effects, yet still cure as many patients as possible. New chemotherapy combinations of as many as 10 different drugs are being studied. The reasoning behind this approach is that even though more drugs are needed, by using less of each drug, fewer side effects may occur. Another approach is using drugs that better target Hodgkin disease cells, as opposed to chemotherapy drugs. Some of these are described below.

The same is true for radiation therapy. Doctors are looking to see which patients might do just as well with lower doses of radiation, or even no radiation. Doctors are also studying whether newer forms of radiation therapy, such as intensity-modulated radiation therapy (IMRT) and proton therapy, might be useful in Hodgkin disease. These approaches focus radiation more precisely on tumors, which limits the doses reaching nearby normal tissues.

Chemotherapy: New chemotherapy drugs and drug combinations are being studied in patients with Hodgkin disease. Some drugs, such as vinorelbine, idarubicin, bendamustine, and gemcitabine, which are already used to treat other cancers, have shown promise against Hodgkin disease that has relapsed after other chemotherapy treatments. Studies are in progress to see if these drugs could be more effective than the ones now in use.

Targeted therapy: Newer drugs that work differently from typical chemotherapy drugs are now being studied as well. These are known astargeted therapy drugs.

For example, a class of drugs known as mTOR inhibitors has shown some promise in early clinical studies against relapsed Hodgkin disease. Drugs called histone deacetylase (HDAC) inhibitors, such as panobinostat, have also shown some early promise.

Other drugs being studied include lenalidomide (Revlimid®) and bortezomib (Velcade®). These drugs are more often used to treat multiple myeloma and some non-Hodgkin lymphomas, but they may prove to be useful in Hodgkin disease as well.

Some newer targeted drugs, such as PLX3397, affect tumor cells other than the lymphoma cells themselves. These other cells actually make up much of the Hodgkin disease tumors and are thought to help the lymphoma cells grow. Research on these types of drugs is still early.

Monoclonal antibodies: Antibodies are proteins normally made by the immune system to help fight infections. Each antibody attacks only a specific target (usually a protein on the surface of an unwanted cell). Monoclonal antibodies are man-made versions of these immune system proteins. Some can kill cancer cells by themselves. Others have radioactive molecules or cell poisons attached to them, which help kill the cancer cells. An advantage of these drugs is that they seem to target lymphoma cells while having fewer side effects than chemotherapy drugs. They may be used alone or combined with chemotherapy.

Some monoclonal antibodies, such as brentuximab vedotin (Adcetris™) and rituximab (Rituxan®), are already being used to treat Hodgkin disease, while others are now being studied.

Hodgkin disease cells have the CD30 molecule on their surface, which is the target of brentuximab vedotin and several other monoclonal antibodies, including XmAb2513.

The cells in the nodular lymphocyte predominant type of Hodgkin disease often have the CD20 antigen, which is the target of rituximab. Studies are now being done to see if rituximab can help treat classic forms of Hodgkin disease as well.

Tositumomab (Bexxar®) is an anti-CD20 antibody that is attached to a radioactive molecule. When injected into the blood, the antibody brings the radiation directly to the lymphoma cells. This drug is used to treat some types of non-Hodgkin lymphoma, but studies are in progress to see if it might also help treat Hodgkin disease.

 

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