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Cancer Medicine :: Lymphoma - Hodgkin - Childhood

Lymphoma - Hodgkin - Childhood

Treatment Options

In general, cancer in children is uncommon, so it can be hard for doctors to plan treatments unless they know what has been most effective in other children. That’s why more than 60% of children with cancer are treated as part of a clinical trial. Clinical trials are research studies that compare the standard treatments (the best proven treatments available) with newer treatments that may be more effective. Investigating new treatments involves careful monitoring using scientific methods, and all participants are followed closely to track their health and progress.

To take advantage of these newer treatments, all children with cancer should be treated at a specialized cancer center. Doctors at these centers have extensive experience in treating children with cancer and have access to the latest research. A doctor who specializes in treating children with cancer is called a pediatric oncologist. In many cases, a team of doctors works with a child and the family to provide care; this is called a multidisciplinary team.

Pediatric cancer centers often have extra support services for children and their families, such as child life specialists, nutritionists, social workers, and counselors. Special activities and programs to help your child and family cope may also be available. An increasing number of pediatric cancer centers also have services for teenagers and young adults. Sometimes, adult cancer centers also offer special services and clinical trials for teens and young adults with cancer.

Treatment overview

Treatment of Hodgkin lymphoma consists of chemotherapy and/or radiation therapy. Surgery is not commonly used as a treatment, although it may sometimes be used for localized nodular lymphocyte predominant Hodgkin lymphoma when the doctor believes it can be completely removed by surgery.

Treatment options and recommendations depend on several factors, including the type and stage of cancer, its risk grouping, possible side effects, and the patient’s preferences and overall health. The amount and type of treatment used to treat Hodgkin lymphoma also depends on how many lymph node areas are involved and how large the lymph nodes have grown. Children with more widespread (advanced) or "bulky" disease may have more cycles of chemotherapy and radiation therapy than children with early-stage disease. Ongoing studies of childhood Hodgkin lymphoma are trying to further reduce the amount of treatment to avoid long-term side effects.

Descriptions of the most common treatment options for childhood Hodgkin lymphoma are listed below. 

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells, usually by stopping the cancer cells’ ability to grow and divide. Systemic chemotherapy is delivered through the bloodstream to reach cancer cells throughout the body. Chemotherapy for childhood Hodgkin lymphoma is given by a pediatric oncologist. A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a set period of time. A patient may receive one drug at a time or combinations of different drugs at the same time.

For children and adolescents, chemotherapy is almost always used. In the past, treatments for Hodgkin lymphoma were one of two combinations of chemotherapy, one called ABVD and another called MOPP. ABVD includes the drugs doxorubicin (Adriamycin), bleomycin (Blenoxane), vinblastine (Velban, Velsar), and dacarbazine (DTIC-Dome). MOPP includes the drugs mechlorethamine (Mustargen), vincristine (Vincasar), prednisone (multiple brand names), and procarbazine (Matulane). These combinations are no longer the standard of care for children because other drugs with fewer long-term side effects are now available, but ABVD may be used for some patients.

Newer treatment combinations for children include many of these drugs but may replace mechlorethamine with cyclophosphamide (Cytoxan, Clafen, Neosar) and often replace procarbazine with etoposide (VePesid, Toposar) to reduce the risk of infertility (the inability to conceive a child or maintain a pregnancy). More recently, treatment plans for later-stage disease have been designed to improve effectiveness by using more intensive combinations of drugs (dose-dense) over shorter periods of time (therapies with combinations of drugs called ABVE-PC, Stanford V, and BEACOPP).

Doctors may recommend treatment with chemotherapy alone or a combination of chemotherapy and radiation therapy for a child with Hodgkin lymphoma. For later-stage disease, current clinical trials often include radiation therapy (see below). For earlier-stage lymphoma, many research studies do not include radiation therapy for children whose disease is treated successfully with chemotherapy. Current chemotherapy regimens evaluate the lymphoma’s response to treatment early in the treatment schedule. A child with lymphoma that responds more quickly to treatment may need less treatment than children with lymphoma that responds to treatment more slowly.

For children with bulky or advanced disease, many doctors feel that combination treatment gives the best chance for cure because there are two ways to attack the cancer cells. In combination treatment, doctors reduce the total amount of chemotherapy and radiation therapy, which should reduce long-term side effects. The most important consideration is to use enough treatment to cure the disease with the first treatment plan. This is because the intensity of therapy is high if the disease recurs.

Because chemotherapy attacks rapidly dividing cells, including those in normal tissues such as the hair, lining of the mouth, intestines, and bone marrow, children receiving chemotherapy may lose their hair, develop mouth sores, or have nausea and vomiting. In addition, chemotherapy may lower the body’s resistance to infection, lead to increased bruising and bleeding, and cause fatigue. These side effects usually can be controlled during treatment and go away after chemotherapy is completed. The severity of the side effects depends on the type and amount of the drug being given and the length of time the child receives the drug. 

The medications used to treat cancer are continually being evaluated. Talking with your child’s doctor is often the best way to learn about the medications prescribed for him or her, their purpose, and their potential side effects or interactions with other medications. 

Radiation therapy

Radiation therapy is the use of x-rays or other particles to kill cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation therapy given from a machine outside the body. A radiation therapy regimen (schedule) usually consists of a specific number of treatments given over a set period of time.

In the past, treatments for Hodgkin lymphoma used high doses of radiation therapy to all of the lymph node areas. Children treated this way had problems with muscle and bone growth and had a higher risk of second cancers as they got older. Today, treatment with radiation therapy alone is never used for children. Much more commonly, doctors combine chemotherapy (see above) with low-dose radiation therapy given to areas where the lymph nodes contain cancer cells. This approach reduces the amount of radiation therapy to the body compared with traditional regimens.

The need for radiation therapy is determined by the stage of disease and whether chemotherapy was effective. Clinical trials are currently in progress to identify patients whose disease can be treated successfully using chemotherapy alone ). However, radiation therapy is a very effective treatment for Hodgkin lymphoma and plays a major role in curing the disease.

In general, short-term side effects from radiation therapy include tiredness, sore throat, dry mouth, mild skin reactions, upset stomach, and loose bowel movements. Long-term side effects of radiation therapy may include growth problems of bones and soft tissues; dental, thyroid, heart, and lung problems; and second cancers. In particular, girls treated for Hodgkin lymphoma with radiation to the mediastinum are at increased risk of breast cancer. 

Palliative/supportive care 

Cancer and its treatment often cause side effects. In addition to treatment to slow, stop, or eliminate the cancer, an important part of cancer care is relieving a person’s symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs.

Palliative care can help a person at any stage of illness. People often receive treatment for the cancer and treatment to ease side effects at the same time. In fact, patients who receive both often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.

Before treatment begins, talk with your child’s health care team about the possible side effects of your child’s specific treatment plan and supportive care options. And during and after treatment, be sure to tell your child’s doctor or another health care team member if your child is experiencing a problem so it can be addressed as quickly as possible.

Recurrent Hodgkin lymphoma

A remission is when cancer cannot be detected in the body and there are no symptoms. This may also be called “no evidence of disease” or NED. 

A remission can be temporary or permanent. This uncertainty leads to many survivors and families feeling worried or anxious that the cancer will come back. While many remissions are permanent, it’s important to talk with your child’s doctor about the possibility of the cancer returning. Understanding the risk of recurrence and the treatment options may help you feel more prepared if the cancer does return. 

If the cancer does return after the original treatment, it is called recurrent cancer. The disease can come back in the same area in which it began or in a new area of the body.

When this occurs, a cycle of testing will begin again to learn as much as possible about the recurrence. After testing is done, you and your child’s doctor will talk about your treatment options. Often the treatment plan will include the therapies described above (such as chemotherapy and radiation therapy) but may be used in a different combination or given at a different pace. Your child’s doctor may also suggest clinical trials that are studying new ways to treat this type of recurrent cancer.

Treatment for recurrent Hodgkin lymphoma depends on where the disease recurs, the type of treatment the child has had previously, and the time since the first treatment was completed. For example, if chemotherapy was given initially, then the child may be given another round of chemotherapy using different drugs. Ifosfamide (Cyfos, Ifex, Ifosfamidum) and vinorelbine (Navelbine) have recently been shown to be an effective treatment for recurrent disease in children and adolescents and are now the standard drugs recommended before stem cell transplantation/bone marrow transplantation (see below) in the Children’s Oncology Groupstudies. The combination of gemcitabine (Gemzar) with vinorelbine is also quite effective in children and adolescents.

If the disease has come back very soon after the first treatment or after the use of chemotherapy and radiation therapy, more aggressive therapy, including bone marrow/stem cell transplantation, may be recommended to increase the chances of keeping the disease in remission.

When cancer recurs, patients and their families often experience emotions such as disbelief or fear. Families are encouraged to talk with their health care team about these feelings and ask about support services to help them cope. 

Stem cell transplantation/bone marrow transplant

Often when high doses of chemotherapy or radiation therapy are used to treat recurrent Hodgkin lymphoma, the bone marrow becomes damaged and can’t produce healthy blood cells. To replace those lost cells, a stem cell transplant may be recommended.

A stem cell transplant is a medical procedure in which diseased bone marrow is replaced by highly specialized cells, called hematopoietic stem cells. Hematopoietic stem cells are found both in the bloodstream and in the bone marrow. Today, this procedure is more commonly called a stem cell transplant, rather than bone marrow transplant, because it is the blood stem cells that are typically being transplanted, not the actual bone marrow tissue.

Before recommending transplantation, doctors will talk with the patient about the risks of this treatment and consider several other factors, such as the type of cancer, results of any previous treatment, and the patient’s age and general health.

There are two types of stem cell transplantation, depending on the source of the blood stem cells: allogeneic (ALLO) and autologous (AUTO). An AUTO transplant is most commonly used for Hodgkin lymphoma. ALLO transplants are not used as frequently for patients with recurrent Hodgkin lymphoma because of the greater risks of serious side effects.

In both types, the goal of transplantation is to destroy cancer cells in the marrow, blood, and other parts of the body and have replacement blood stem cells to create healthy bone marrow. In most stem cell transplants, the patient is treated with high doses of chemotherapy and/or radiation therapy to destroy as many cancer cells as possible.

If treatment fails

Although treatment is successful for the majority of children with Hodgkin lymphoma, sometimes it is not. If a child’s cancer cannot be cured or controlled, this is called advanced or terminal cancer. This diagnosis is stressful, and it may be difficult to discuss. However, it is important to have open and honest conversations with your child’s doctor and health care team to express your family’s feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families.

Parents or guardians are encouraged to think about where the child would be most comfortable: at home, in a home-like setting elsewhere, in the hospital, or in a hospice environment. Nursing care and special equipment can make staying at home a workable alternative for many families. Some children may be happier if they can arrange to attend school part-time or keep up other activities and social connections. The child’s health care team can help parents or guardians decide on an appropriate level of activity. Making sure a child is physically comfortable and free from pain is extremely important as part of end-of-life care. 

Clinical Trials

Doctors and scientists are always looking for better ways to treat children with Hodgkin lymphoma. To make scientific advances, doctors create research studies involving people, called clinical trials.

Many clinical trials are focused on new treatments, evaluating whether a new treatment is safe, effective, and possibly better than the current (standard) treatment. These types of studies evaluate new drugs, different combinations of existing treatments, new approaches to radiation therapy or surgery, and new methods of treatment. Children who participate in clinical trials are often among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.

There are also clinical trials that study new ways to ease symptoms and side effects during treatment and manage the late effects that may occur after treatment. Talk with your child’s doctor about clinical trials regarding side effects. In addition, there are ongoing studies about ways to prevent the disease.

Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that these studies are the only way to make progress in treating Hodgkin lymphoma. Even if they do not benefit directly from the clinical trial, their participation may benefit future children with Hodgkin lymphoma.

Sometimes people have concerns that, by participating in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” The use of placebos in cancer clinical trials is rare in general, and placebos are never used in clinical trials for treating children with cancer. When a placebo is used in a study, it is done with the full knowledge of the participants. 

To join a clinical trial, parents and children must participate in a process known as informed consent. During informed consent, the doctor should list all of the patient’s options so that the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different from the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment. 

Coping with side Effects

Fear of treatment side effects is common after a diagnosis of cancer, but it may help to know that preventing and controlling side effects is a major focus of your child’s health care team. This is called palliative or supportive care, and it is an important part of the overall treatment plan, regardless of the stage of disease.

Common side effects from each treatment option for Hodgkin lymphoma are described in detail within the Treatment section. Side effects depend on a variety of factors, including the cancer’s stage, the length and dosage of treatment(s), and your child’s overall health.

Before treatment begins, talk with your child’s doctor about possible side effects of each type of treatment you/your child will be receiving. Ask which side effects are most likely to happen, when they are likely to occur, and what can be done to prevent or relieve them. And, ask about the level of caregiving your child may need during treatment and recovery, as family members and friends often play an important role in the care of a person with Hodgkin lymphoma. 

In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. Patients and their families are encouraged to share their feelings with a member of their health care team who can help with coping strategies. 

During and after treatment, be sure to tell the health care team about the side effects your child experiences, even if you feel they are not serious. Sometimes, side effects can last beyond the treatment period, called a long-term side effect. A side effect that occurs months or years after treatment is called a late effect. Treatment of both types of effects is an important part of survivorship care. 

After Treatment

After treatment for Hodgkin lymphoma ends, talk with your child’s doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your child’s recovery for the coming months and years. All children treated for cancer, including Hodgkin lymphoma, should have life-long, follow-up care.

People with Hodgkin lymphoma are most often adolescents or young adults. While the treatment period is limited and the outcome is often excellent, there are several possible long-term side effects. The long-term side effects of chemotherapy also depend on the type and total dose of each drug. These include heart problems (after doxorubicin or radiation therapy), lung problems (after bleomycin or radiation therapy), thyroid problems (after radiation therapy), secondary cancers (after radiation therapy or chemotherapy), and reproductive effects (after procarbazine, mechlorethamine, or pelvic radiation). In addition, children who had a splenectomy (surgical removal of the spleen) have an ongoing chance of serious infection. Rarely, children with Hodgkin lymphoma develop a second cancer, acute myeloid leukemia, because of chemotherapy’s effects on bone marrow function. Fortunately, the risk of long-term side effects is much lower with newer treatment plans that limit the doses of drugs that cause serious health problems.

For most Hodgkin lymphoma survivors, the medical side effects of treatment do not significantly affect life span. However, Hodgkin lymphoma survivors report significant concerns regarding their health status compared with other survivors of childhood cancer. This may result from the social and emotional effects of treatment during adolescence, when the adolescent may feel different from healthy peers. In addition, many Hodgkin lymphoma survivors experience long-term fatigue that may require lifestyle changes, such as taking a reduced course load at college or choosing employment that is consistent with the individual’s energy level. 

Infertility may also affect the young adult who is hoping to someday become pregnant or father a child. Newer reproductive technologies may help some of these individuals; current treatment plans also attempt to reduce exposure to alkylating agents to limit such risks

Based on the type of treatment the child received, the doctor will determine what examinations and screening tests are needed to check for long-term side effects and the possibility of secondary cancers. Follow-up care should also address the child’s quality of life, including any developmental or emotional concerns. 

The child’s family is encouraged to organize and keep a record of the child’s medical information. That way, as the child enters adulthood, he or she has a clear, written history of the diagnosis, the treatment given, and the doctor’s recommendations about the schedule for follow-up care. The doctor’s office can help you create this. This information will be valuable to doctors who care for your child during his or her lifetime. 

Children who have had cancer can also enhance the quality of their future by following established guidelines for good health into and through adulthood, including not smoking, maintaining a healthy weight, eating a balanced diet, and participating in regular physical activity. Talk with the doctor about developing a plan that is best for your child’s needs.  

Ongoing follow-up care by health care professionals experienced in long-term side effects is important. Preventive health care, including breast cancer screening (after mediastinal radiation therapy), not smoking (after bleomycin or radiation therapy due to enhanced lung cancer risk), and reducing the risk for heart disease through exercise, diet, and medication are important steps for Hodgkin lymphoma survivors. Such preventative measures may foster better, long-term outcomes and offer the person some control of his or her own health.

Latest Research

Doctors are working to learn more about Hodgkin lymphoma, ways to prevent it, how to best treat it, and how to provide the best care to people diagnosed with this disease. The following areas of research may include new options for patients through clinical trials. Always talk with your child’s doctor about the diagnostic and treatment options best for your child.

New drugs. To reduce side effects and improve the effectiveness of treatment, it is important for researchers to find new drugs, as well as new combinations of current drugs.

ALLO stem cell transplantation. As explained in the Treatmentsection, stem cell transplantation is sometimes used to treat Hodgkin lymphoma. Doctors are researching increasing the use of stem cell transplantation, including using donor blood stem cells to replace the patient’s bone marrow (called allogeneic or ALLO transplantation).

Personalized treatments. Overall, the major goal of Hodgkin lymphoma research is to improve treatment effectiveness while reducing long-term side effects. Early response of the disease to a specific treatment is being studied as a method to tailor therapy to the individual. Shorter, more intensive regimens may improve effectiveness. It is important that researchers learn which patients need radiation therapy and which patients may be cured without the long-term side effects of radiation exposure.

Targeted therapy. Targeted therapy is a treatment that targets specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. Recent studies are evaluating antibodies—such as brentuximab vedotin (SGN-35)—directed against proteins on the Hodgkin lymphoma cells. .

Supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current Hodgkin lymphoma treatments in order to improve patients’ comfort and quality of life.

 

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