Patient information: Staging and treatment for Hodgkin's disease DEFINITION — Hodgkin's disease is a form of malignant lymphoma, a type of cancer of the body's lymphatic system. As such, it is sometimes referred to as Hodgkin's lymphoma. The lymphatic system consists of a network of channels and vessels that contain a special fluid (lymph) that helps the body fight infection. The fluid drains into areas known as lymph nodes which are located throughout the body (such as in the armpits, along the neck, and in the groin). Other organs in the body that are part of the lymphatic system are the spleen, bone marrow, tonsils, and thymus. In Hodgkin's disease, cancer cells of a specific type (called Hodgkin's Reed-Sternberg cells) begin to grow in the lymphatic system. They interfere with the normal functioning of the lymphatic system, and can collect in masses in various areas of the body. Fortunately, Hodgkin's disease is now one of the most treatable forms of cancer. About 75 percent of patients diagnosed with Hodgkin's disease can be cured with treatment. Over 90 percent are expected to live at least 10 years following diagnosis and treatment. One of the reasons for this success is the ability of doctors to carefully plan treatments based on a patient's stage of illness. "Staging" a patient's Hodgkin's disease is the first important step in identifying the most appropriate course of treatment. STAGING — Research on Hodgkin's disease has enabled doctors to carefully divide patients into groups, or stages, based on various criteria present at the time of diagnosis. Treatment decisions are based in large part on the stage of disease that is found. The following are definitions of terms related to the staging criteria:
Stage I — Only one lymph node region is involved, or only one lymph structure is involved. Stage II — Two or more lymph node regions or lymph node structures on the same side of the diaphragm are involved. Stage III — Lymph node regions or structures on both sides of the diaphragm are involved. Stage IV — There is widespread involvement of a number of organs or tissues other than lymph node regions or structures, such as the liver, lung, or bone marrow. Subclassifications — Additional criteria help doctors further identify subgroups within each stage, as follows: The presence or absence of the symptoms of unexplained fever, night sweats, or unexplained weight loss during the six months prior to diagnosis is an important part of the staging process. The letter "A," as in stage IIA, means that these symptoms were absent. The letter "B," as in stage IIIB, means that these symptoms were present. The presence of local spread of the disease from one nodal area or structure to surrounding or attached tissue in the same area of the body is indicated by the letter "e," as in stage IIe. Involvement of the spleen is designated by the letter "s", as in stage IIIs. The presence of "bulky disease," referring to larger masses of cancerous tissue, is indicated by the letter x. For example, a patient with Hodgkin's disease involving lymph nodes in the neck, mediastinum, and groin (ie, involvement above and below the diaphragm) who also has symptoms of fever, night sweats, and weight loss (ie, systemic symptoms), would be in stage IIIB. DETERMINING STAGE — A number of tools are available to the doctor during the staging process. Not all of these tools are needed in every case in order to reliably determine the stage of disease. History and physical exam — A careful history will help the doctor determine whether or not symptoms related to Hodgkin's disease have been present. The physical exam may reveal swollen lymph nodes in various locations Diagnostic tests — A number of diagnostic tests are available to help the doctor determine which areas of the body have been affected by Hodgkin's disease. Tests that may be done include:
Staging laparotomy — In the past, surgery was done in order to directly visualize and take samples from areas in the abdomen in order to determine the stage of the Hodgkin's disease. Typically, the spleen was removed during this procedure. The operation, called a staging laparotomy, is now rarely done, because other improved methods that do not involve the risks of surgery have improved the accuracy of staging. In addition, the availability of less toxic chemotherapy and radiation therapy and the use of both modalities in early stage Hodgkin's disease, have reduced the need for the more precise surgical staging. However, staging laparotomy may still be used in patients who have early stage Hodgkin's disease with a good prognosis, because doctors may want to use only minimal treatment. Staging laparotomy provides reassurance that the disease is not widespread, and gives doctors and patients the option of forgoing some of the toxicities associated with more extensive treatment. TREATMENT — The mainstays of treatment for Hodgkin's disease are chemotherapy and radiation. Chemotherapy uses strong medicines to kill cancer cells, radiation uses carefully measured doses of radiation. Specific side effects of chemotherapy and radiation, as well as typical treatment schedules, are discussed with the patient before treatment. Decisions about whether to use chemotherapy, radiation, or both depends in large part on the stage of the disease. The patient's preferences in terms of risks and benefits are also an important part of the decision. Stage I or II — For patients with Stage I or II Hodgkin's disease, doctors may perform an additional analysis in order to determine the best treatment choices. Research has shown that patients at these early stages of Hodgkin's disease can be further divided into favorable, and unfavorable categories. An unfavorable designation indicates the likelihood of more serious disease, but is still within the earliest stages of Hodgkin's. It is a way to help fine tune treatment for the early stage patient. Criteria used to determine favorable or unfavorable disease at Stage I or II may include factors such as whether or not a large mass is present in the chest, how many lymph node regions are affected by the Hodgkin's disease, the age of the patient, the presence of symptoms, or a specific abnormality in the erythrocyte sedimentation rate (a blood test). Decisions about treatment may vary depending on whether a favorable or unfavorable pattern of disease is found. Favorable disease — A number of choices are available for patients with stage I or II disease with a favorable designation. As noted above, some of these patients may undergo staging laparotomy in order to be sure that the disease has not spread. Once this has been confirmed, patients may be treated with radiation only to the affected area. In patients who do not undergo laparotomy, treatment choices include chemotherapy and radiation, or radiation alone. The use of chemotherapy along with radiation is associated with a lower relapse rate, but potentially higher toxicity. Radiation alone carries a higher risk of relapse, but patients who do relapse may be easier to treat than those who relapse following chemotherapy and radiation. It is extremely important that patients and doctors discuss these options thoroughly, as the patient's preferences in terms of risk of recurrence of disease versus possible toxic effects of treatment are very important parts of the decision-making process. Unfavorable disease — Patients at Stage I or II with an unfavorable designation generally are treated with both chemotherapy and radiation. Stage IIIA — Patients with Stage IIIA Hodgkin's can achieve initial cure rates of 60 to 80 percent with radiation therapy of all the lymph nodes and no chemotherapy. However, because of the high recurrence rate of up to 50 percent with this approach, chemotherapy is usually given as well. In some cases, these patients are treated with chemotherapy alone. The decision is influenced by the patient's preferences in terms of duration of treatment and expected benefits and toxicities. Some clinical trials have demonstrated reduced recurrence rates with chemotherapy and radiation therapy compared to chemotherapy alone, but none of the trials have shown survival differences. Stage IIIB — Patients with stage IIIB disease are usually treated with chemotherapy alone. In some cases, radiation therapy may be used in areas with large masses or in areas that do not show a complete response to chemotherapy. Stage IV — Treatment is similar to that seen in stage IIIB, with chemotherapy alone being the usual approach. Radiation therapy may be used in selected instances. Chemotherapy programs — Chemotherapy is usually given with a combination of medicines, each one of which may attack the malignant cells by a different route. These programs, examples of which include MOPP, ABVD, and Stanford V, may have differing degrees of effectiveness, and their short-term as well as long-term side effects may also differ. Your physicians will discuss these differences with you, should chemotherapy be an option. Bone marrow transplantation — High dose chemotherapy followed by autologous stem cell rescue is an effective treatment for patients whose disease has recurred after initial chemotherapy or chemotherapy and radiation therapy. Bone marrow transplantation (also called hematopoietic cell transplantation) is a treatment in which very high doses of chemotherapy or radiation are used, doses that kill cancer cells, but that also destroy very young blood cells (stem cells) forming in the bone marrow. This leaves the body temporarily unable to produce needed red and white blood cells and platelets. New stem cells are transplanted to the patient in order to restore the blood production function of the bone marrow. |