Hodgkin's Disease

Description

Treatment Options by Stage

Treatment is guided by the stage of the disease and usually relies on the location and extent of the disease. Treatment may vary within a stage, depending on whether it is categorized as either A or B. (Systemic symptoms are absent in "A" and present in "B.) The presence of B symptoms increases the risk of relapse, and so may require more aggressive treatments for that stage.

Early Stages (I or II). For non- disease in stages I or II, the following treatments may be used:

• Treatment in Adults. Physicians usually recommend radiation first for adults with HD. It provides excellent remission rates, although studies have reported a number of serious long-term complications in some patients. Selected patients in early stages may also be candidates for radiation limited only to areas above the diaphragm (called the mantle field), which can also have excellent results although still poses a considerable risk for late serious complications.
• Treatment in Children. Chemotherapy and low-dose radiation is the standard treatment for most children and adolescents who have not reached full growth. Specific chemotherapy combinations have been developed to reduce the risks for infertility, leukemia, and toxic effects on the heart and lungs. Researchers are studying the use of chemotherapy alone in this group.

Later Stages. For stage III disease, chemotherapy, often with radiation, is a standard treatment. For stage IV disease, chemotherapy alone is generally recommended. The latest chemotherapy regimens are achieving survival rates that reach 90%.

Relapse. Relapse after treatment occurs in 20% to 35% of patients. Treatments for relapse include chemotherapy, radiation, and bone marrow or blood stem cell transplantation. Many patients respond favorably to such treatments, although another relapse is still possible.

Click the icon to see an illustrated series detailing bone marrow transplant surgery.

Stage I Hodgkin's Disease

Disease is limited to a single node region (I) or has involved one neighboring area or a single nearby organ (IE). The standard treatment for stage I disease is usually radiation for adult patients who have determined the stage using pathologic staging with laparotomy. Chemotherapy with low-dose radiation is now the standard approach for children and adolescents. Cure rates can be greater than 90%.

Stage IA. Treatments depend on location:

• Malignancy is above the diaphragm and does not involve a large part of the chest: 1. Radiation therapy to the mantle field (chest, neck, and arm pits) and to the lymph nodes in the upper abdomen and spleen. 2. Radiation therapy to a mantle field in certain patients (best candidates are females with nodular sclerosis or lymphocyte predominant cell types, who are no older than 40 years, have no "B" symptoms, and have erythrocyte sedimentation rate (ESR) levels less than 50). 3. Radiation therapy to a mantle field, the lymph nodes in the upper abdomen, and the spleen (subtotal node irradiation). 4. Chemotherapy alone is under investigation.
• Malignancy is bulky (above the diaphragm but involves a large part of the chest). 1. Chemotherapy plus radiation therapy to a mantle field is a common approach.
• Malignancy is below the diaphragm: 1. Chemotherapy with or without radiation therapy. 2. Radiation therapy to the lymph nodes in the upper abdomen and pelvis or total nodal irradiation (which includes these regions plus the mantle field). The spleen or the groin may also be treated if needed.

Stage IB. Treatments depend on location:

• Malignancy is above the diaphragm and does not involve a large part of the chest: 1. Chemotherapy plus radiation therapy to a mantle field (in patients who have severe symptoms and did not undergo laparotomy to determine the extent of the disease below the diaphragm). 2. Radiation therapy to the mantle field and to the lymph nodes in the upper abdomen is sometimes considered, but relapse rate can be high if significant B symptoms are present. 3. Chemotherapy alone under investigation for children.
• Malignancy is bulky (above the diaphragm but involves a large part of the chest): 1. Chemotherapy plus radiation therapy to a mantle field is a common approach.
• Malignancy is below the diaphragm, the treatment may be one of the following: 1. Chemotherapy with or without radiation therapy to the upper abdomen and pelvis, to the areas that contain cancer, or to the spleen. 2. Total nodal irradiation or radiation to lymph nodes in the upper abdomen and pelvis.

Stage II Hodgkin's Disease

Disease is limited to two or more lymph nodes on the same side of the diaphragm (II) or involvement of a single neighboring organ or area and one or more nearby lymph nodes; other lymph nodes on the same side of the diaphragm may be involved (IIE).

There are few differences between treatments for State IIA and IIB and the approach for both depends on the extent and location of the disease:

• Nonbulky disease. Standard treatments are usually radiation alone for adult and possibly adolescent (especially male) patients and chemotherapy with low-dose radiation for children.
• Malignancy is above the diaphragm and does not involve a large part of the chest. The treatment may be one of the following: 1. Radiation therapy to a mantle field and to the lymph nodes in the upper abdomen. 2. Radiation therapy to a mantle field only (in certain patients). (See Stage I above.) 3. Chemotherapy alone or with radiation therapy (combined modality) being evaluated for those with non-bulky stage IIA. 4. Under investigation: Radiation therapy to a mantle field only in patients with lymphocyte predominant cell types, who are no older than 40 years.
• Malignancy is above the diaphragm but involves a large part of the chest. 1. Chemotherapy plus radiation therapy to a mantle field is a common approach
• If the malignancy is below the diaphragm, the treatment may be one of the following: 1. Chemotherapy alone or with radiation therapy (combined modality). 2. Radiation therapy to the lymph nodes in the upper abdomen and pelvis. The spleen may also be treated if needed. 3. Total nodal irradiation.

Stage III Hodgkin's Disease

Disease is in lymph nodes on both sides of the diaphragm (III), which may also be accompanied by localized involvement of an associated organ or site outside the lymph node (IIIE), by involvement of the spleen (IIIS), or by both (IIIE+S). In addition, Stage III may be further categorized by the extent of its spread into the spleen or where it has spread in the abdominal area. Survival rates in some cases can be as high as 90%.

Stage IIIA. Chemotherapy is the most common treatment approach for most adults and children. Radiation may be added under certain circumstances, especially to provide localized treatment of bulky areas. (Radiation does not appear to offer any survival advantage for patients whose disease is in complete remission after chemotherapy.)

• The malignancy does not involve a large part of the chest: 1. Chemotherapy alone. 2. Chemotherapy with radiation therapy (combined modality). 3. Total or subtotal nodal radiation therapy alone in adults if disease is only in the upper abdomen and fewer than five nodes in the spleen are affected, although relapse rate may be high.
• The malignancy involves a large part of the chest: 1. Standard chemotherapy regimens. 2. Chemotherapy plus radiation therapy (combined modality). 3. Investigative treatments.

Stage IIIB. Chemotherapy alone is the standard treatment for most adults and children. Radiation is often added to treat areas of bulky tumor. 1. Chemotherapy. 2. Chemotherapy and radiation to areas where the cancer is found or to more extended areas. (Radiation does not appear to offer any survival advantage for patients whose disease is in complete remission after chemotherapy.)

Stage IV Hodgkin's Disease

Disease has spread to organs outside the lymph system, such as liver, lung, or bone marrow. Even in this population, high long-term survival rates of over 85% are possible, including in children.

Treatment may be one of the following: 1. Chemotherapy alone. 2. Chemotherapy with limited radiation to places of bulky disease. 4. A clinical trial of novel chemotherapy regimens or of stem-cell transplantation.

Click the icon to see an image of liver involvement in Hodgkin's disease.

Relapsed or Refractory Hodgkin's Disease

When disease recurs or persists after initial treatment either in the same area or in another part of the body, the next round of therapy depends on where the disease returns and the previous treatment used.

• If the previous treatment was radiation therapy without chemotherapy, salvage chemotherapy is the usual choice.
• If the patient was previously treated with chemotherapy, then the choice may be radiation therapy to the lymph nodes with or without salvage chemotherapy.
• In some patients, if the disease has persisted or if relapse has occurred after chemotherapy with or without radiation, high-dose chemotherapy and stem cell transplantation may be given.