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Hodgkins Disease

What is Hodgkin's disease?

Hodgkin's disease is a type of lymphoma. Lymphomas are cancers that develop in the lymph system, part of the body's immune system.

The lymph system is made up of thin tubes that branch, like blood vessels, into all parts of the body. Lymph vessels carry lymph; a colorless, watery fluid that contains white blood cells called lymphocytes. Along the network of vessels are groups of small, bean-shaped organs called lymph nodes. The lymph nodes make and store infection-fighting white cells. They are the "swollen glands" in the neck of a child with Strep Throat, showing that the normal role of lymph nodes is to help the body to respond to infection. Clusters of lymph nodes are found in the underarm, pelvis, neck, chest and abdomen. The spleen (an organ in the upper abdomen that makes lymphocytes and filters old blood cells from the blood, the thymus (a small organ beneath the breastbone), and the tonsils (an organ in the throat) are also part of the lymph system.

Because there is lymph tissue in many parts of the body, Hodgkin's disease can start in almost any part of the body. The cancer can spread to any organ or tissue in the body, including the liver, bone marrow (the spongy tissue inside the large bones of the body that makes blood cells), and the spleen.

Lymphomas are divided into two general types: Hodgkin's lymphoma (disease) and non-Hodgkin's lymphomas. Hodgkin's disease is mostly found in individuals in their mid to late 20's or in late adulthood. Hodgkin's disease is rare in children under 5 years of age. In children under age 10, it is more common in boys than girls. The symptoms of childhood Hodgkin's disease may include any of the following: painless swelling of the lymph nodes in the neck or underarm area that doesn't go away; fever that doesn't go away; night sweats; and weight loss without dieting. If your child has any of these symptoms, take them to their doctor. If the lymph nodes don't feel normal when examined, their doctor may consult a pediatric oncologist or surgeon. The surgeon may need to cut out a small piece of tissue from one of the swollen lymph nodes. This is called a biopsy. The tissue will be examined under a microscope to look for cancer cells.

The chance of recovery (prognosis) is dependent on the stage of the cancer (whether it is just in one area or has spread throughout the body), the age and overall condition of the child. More than 90% of children and adolescents with Hodgkin's disease are cured with chemotherapy with or without low dose radiation therapy.

Stages of childhood Hodgkin's disease

Once childhood Hodgkin's disease is diagnosed, more tests will be done to find out if the cancer has spread to other parts of the body. This is called staging. It is important to know the stage to plan treatment. Treatment should be planned by a pediatric oncologist with experience in treating cancers of childhood and adolescence.

The stage of disease will be determined by physical examination, blood tests, bone marrow testing, and different kinds of x-rays (including CT scans, Gallium scans and PET scans). This is called clinical staging.

Each stage of childhood Hodgkin's disease is further divided into A and B categories based on whether the individual has specific symptoms. Patients with no symptoms are in the A category, while those who have symptoms (unexplained fever, unexplained weight loss of greater than 10% of body weight, drenching night sweats) are in the B category. For example, a child with stage I disease without any symptoms is said to have stage IA disease; a child with stage I disease and symptoms is said to have stage IB disease.

The following stages are used for childhood Hodgkin's disease:

Stage I

Cancer is found in only one lymph node area or in only one area or organ adjacent to the lymph nodes.

Stage II

Either of the following means the disease is stage II:

  • Cancer is found in two or more lymph nodes areas on the same side of the diaphragm (the thin muscle under the lungs that helps you breathe).
  • Cancer is found in only one area or organ outside of the lymph nodes and in the lymph nodes around it. Other lymph node areas on the same side of the diaphragm may also have cancer.
Stage III

Cancer is found in lymph nodes areas on both sides of the diaphragm. The cancer may also have spread to an area or organ near the lymph node areas and/or the spleen.

Stage IV

Any of the following means the disease is stage IV:

  • Cancer is found in more than one spot in an organ or organs outside of the lymph system.
  • Cancer cells may or may not be found in the lymph nodes near these organs.
  • Cancer is found in only one organ outside of the lymph system, but lymph nodes far away from that organ are involved.

Recurrent

Recurrent disease means that the cancer has come back after it has been treated. It may come back in the area where it first started or in another part of the body.

How childhood Hodgkin's disease is treated

There are treatments available for all patients with childhood Hodgkin's disease. The most common treatment involves chemotherapy with or without radiation therapy. Treatment will be different depending on the stage of the cancer and the age of the patient. Bone marrow transplants are being studied in clinical trials for certain high stage patients.

Radiation therapy is the use of high-energy x-rays to kill cancer cells and shrink tumors. Radiation for childhood Hodgkin's disease usually comes from a machine outside the body (external beam radiation therapy). Radiation therapy given to the neck, chest and lymph nodes under the arms is called mantle radiation therapy. Radiation therapy given to the mantle and to the lymph nodes in the upper abdomen, the spleen, and the lymph nodes in the pelvis is called total nodal irradiation. Most pediatric and adolescent patients receive low dose involved field radiation and chemotherapy.

Chemotherapy is the use of drugs to kill cancer cells and shrink tumors. Chemotherapy may be taken by pill, or it may be put into the body by inserting a needle into a vein or muscle. Chemotherapy is called a systemic treatment because the drugs enter the blood stream, travel through the body, and can kill cancer cells through the body.

Bone marrow transplantation is a type of treatment used in certain situations. Sometimes Hodgkin's disease becomes resistant to treatment with radiation therapy or chemotherapy. Very high doses of chemotherapy are then used to treat the cancer. Because the high doses of chemotherapy can destroy the bone marrow, bone marrow (or peripheral blood stem cells) is taken from the patient before treatment. The marrow is then frozen and high-dose chemotherapy with or without radiation therapy is given to the patient. The stored bone marrow is then given back to the patient through a needle into a vein to replace the marrow that was destroyed. This type of transplant is called an autologous transplant. If the marrow given is taken from another person, the transplant is called an allogeneic transplant.

Some patients develop another form of cancer (a second malignancy) as a result of treatment for Hodgkin's disease; therefore, regular follow-up evaluations are necessary. Female patients who receive radiation therapy between the ages of 10 and 16 years have an increased risk of developing breast cancer at an early age.

Treatment by stage and clinical trial

Treatment for childhood Hodgkin's disease depends on the type and stage of disease, how the stage was determined, the child's age, symptoms, and general health.

Patients may receive treatment that is considered standard based on its effectiveness in patients in previous clinical trials, or you or your child may choose to be treated in the context of a clinical trial. Clinical trials are designed to test potentially better treatments with therapy that is currently accepted as standard of care in hope of finding better ways to treat cancer patients. Clinical trials are ongoing in most parts of the country for most stages of childhood Hodgkin's disease. The good news is that Children's Oncology Group clinical trials are closely overseen by a data monitoring committee, so that in real time, if one treatment is found to be better than another, the treating physicians are notified immediately, and your child's treatment will be changed right away if necessary.

The discussion below is detailed in an attempt to help you discuss treatment options with your child's physicians. The country's pediatric oncologists are trying to work together to find out how to use the least amount of therapy to cure childhood and adolescent Hodgkin's Disease. This is an attempt to minimize long term toxicity, including the development of second cancers when the child is older. The recommended therapy will depend on the details of your child's staging evaluation, and on which of the various clinical trials are being offered at your treating institution. The clinical trials will be more alike than different. At Cleveland Clinic Children's, we would be happy to review the recommendation you are given if that would be helpful to you.

Stage I childhood Hodgkin's disease

Treatment depends on whether the disease is stage IA or stage IB and where the cancer is found. This usually involves chemotherapy and low dose involved field radiation therapy. Greater than 90% of patients with stage I Hodgkin's disease will be cured.

Stage IA

If the cancer is above the diaphragm and does not involve a large part of the chest, treatment may be one of the following:

  1. Chemotherapy with low dose radiation therapy to areas that contain cancer.
  2. A clinical trial of chemotherapy with or without radiation therapy.

If the cancer is above the diaphragm, but involves a large part of the chest, treatment may be one of the following:

  1. Chemotherapy followed by radiation therapy to the chest or the mantle.
  2. A clinical trial of chemotherapy plus low-dose radiation therapy.
Stage IB

If the cancer is above the diaphragm and does not involve a large part of the chest, treatment may be one of the following:

  1. Chemotherapy plus low dose radiation therapy to areas that contain cancer.
  2. Treatment is a clinical trial, involving chemotherapy plus low dose radiation therapy.

If the cancer is above the diaphragm, but involves a large part of the chest, treatment may be one of the following:

  1. Chemotherapy plus radiation therapy to the chest or the mantle field.
  2. A clinical trial of chemotherapy followed by low-dose radiation therapy.
Stage II childhood Hodgkin's disease

Treatment depends on whether the disease is stage IIA or stage IIB and where the cancer is found. Greater than 85% of patients with stage II Hodgkin's disease are cured.

Stage IIA

If the cancer is above the diaphragm and does not involve a large part of the chest, treatment may be one of the following:

  1. Chemotherapy plus low dose radiation therapy to areas that contain cancer.
  2. A clinical trial of low dose chemotherapy with or without radiation therapy.
  3. A clinical trial of chemotherapy alone.

If the cancer is above the diaphragm, but involves a large part of the chest, treatment may be one of the following:

  1. Chemotherapy plus low dose radiation therapy to the chest or the mantle.
  2. A clinical trial of chemotherapy plus low dose radiation therapy.
Stage IIB

If the cancer is above the diaphragm and does not involve a large part of the chest, treatment may be one of the following:

  1. Chemotherapy plus low dose radiation therapy to areas that contain cancer.
  2. Treatment in a clinical trial.

If the cancer is above the diaphragm, but involves a large part of the chest, treatment may be one of the following:

  1. Chemotherapy plus low dose radiation therapy to the chest or the mantle.
  2. A clinical trial of chemotherapy followed by low dose radiation therapy.
Stage III childhood Hodgkin's disease

Treatment depends on whether the disease is stage IIIA or stage IIIB.

Stage IIIA

Treatment may be one of the following:

  1. Chemotherapy alone
  2. Chemotherapy plus low dose radiation therapy to areas that contain cancer.
  3. A clinical trial of chemotherapy with or without total nodal irradiation.
Stage IIIB

Treatment may be one of the following:

  1. Chemotherapy alone.
  2. Chemotherapy plus low dose radiation therapy to areas that contain large amounts of cancer.
  3. Treatment in a clinical trial.
Stage IV childhood Hodgkin's disease

Treatment may be one of the following:

  1. Chemotherapy alone.
  2. Chemotherapy plus low dose radiation therapy to areas that contain cancer.
  3. A clinical trial of chemotherapy with or without total lymph nodes radiation.

Recurrent childhood Hodgkin's disease

Almost all pediatric patients with Hodgkin's disease who relapse/recur or progress (on therapy) have received prior chemotherapy and low dose involved field radiation therapy. Patients who have recurrent/refractory/progressive disease will receive treatment that may include high dose chemotherapy and stem cell reconstitution with or without additional radiation therapy.

Cleveland Clinic Children's

Here at Cleveland Clinic Children's, we specialize in the care of children and young adults with cancer. We use a team approach that cares for all members of the family, recognizing that this is a very difficult time for all of them. Our goal is to have our patients get through therapy with as normal a life as possible, and to emerge from therapy knowledgeable about how to maintain their health.

Should you have more questions about the child or young adult in your life with cancer, please contact The Cleveland Clinic Cancer Answer line at 216.444.3024 or 800.223.2273, extension 4-3024. Your question will be forwarded to the Chair of the Pediatric Oncology Department.

We wish you and your child well.

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