Hodgkin's Disease

Definition

Hodgkin's disease is a type of lymphoma. Lymphomas are cancers that develop in the lymphatic system. The job of the lymphatic system is to help fight diseases and infection.

Description

The lymphatic system includes a network of thin tubes that branch, like blood vessels, into the tissues throughout the body. Lymphatic vessels carry lymph, a colorless, watery fluid that contains infection-fighting cells called lymphocytes. Along this network of vessels are groups of small, bean-shaped organs called lymph nodes that filter the lymph as it passes through the nodes. Clusters of lymph nodes are found in the underarm, groin, neck, and abdomen.

Other parts of the lymphatic system are the spleen, thymus, tonsils, and bone marrow. Like all types of cancer, Hodgkin's disease affects the body's cells. Healthy cells grow, divide, and replace themselves in an orderly manner. This process keeps the body in good repair. In Hodgkin's disease, cells in the lymphatic system grow abnormally and can spread to other organs. As the disease progresses, the body is less able to fight infection.

Causes and Risk Factors

Hodgkin's disease is rare. It accounts for less than 1 percent of cancer in this country. It is most often seen in young people aged 15 to 34 and in people over the age of 55. Its cause is unknown.

Symptoms

The symptoms of lymphatic cancer vary from person to person and may easily be confused with noncancerous conditions. In most cases, the first sign of lymphoma is a painless swelling in the neck, armpit or groin, caused by enlarged lymph glands. In non-Hodgkin's lymphoma, the swelling may arise in the abdomen. Some lymphoma patients complain of persistent or recurrent fever, night sweats, fatigue and weight loss. Sometimes, itching of the skin (pruritus) marks the early stages of Hodgkin's disease and other lymphomas.

Although all of these symptoms can be present in other illnesses, they may suggest lymphoma, particularly when lymph-node swelling lasts more than six weeks and does not respond to antibiotics. Still, the only sure way to determine whether lymphoma is present is by examination of lymph tissue surgically removed by biopsy.

Diagnosis

Like most cancers, Hodgkin's disease is best treated when diagnosed early. You should see your doctor if you have one of the following for more than two weeks:

• painless swelling in the lymph nodes of the neck, underarm or groin

• fever that does not go away

• night sweats

• feeling tired all the time

• weight loss without dieting

• itchy skin

If you have symptoms, your doctor will examine you carefully and check for swelling or lumps in your neck, underarms or groin. If the lymph nodes do not feel normal, your doctor may need to do a biopsy.

Treatment

About 7 percent of all newly diagnosed people with Hodgkin's disease can be cured with current radiotherapy and combination chemotherapy.

The most important factors for determining the prognosis and outlining treatment plans are the stages of disease, the presence or absence of symptoms, and the presence of large masses.

Other factors include the patient's age, the extent of splenic disease found during a laparotomy, the extent of abdominal lymph node involvement, and the results of a laboratory test measuring the erythrocyte (red cell) sedimentation rate.

Radiation Therapy and Chemotherapy

Radiation therapy is considered the treatment of choice for those with favorable localized disease - Stage I and Stage II - with no large masses and no symptoms.

For all other patients, chemotherapy is required. Combined radiation and chemotherapy is commonly used for large individual tumor masses, so-called bulky disease. Even patients who have recurrent disease after initial treatment with radiation have an excellent chance for a prolonged disease-free survival with combination chemotherapy. Patients with recurrent disease after chemotherapy have a less favorable prognosis, but long remissions have been reported after more chemotherapy or intensive chemotherapy, and radiation therapy followed by bone marrow transplantation.

The leading clinical investigations in early stage Hodgkin's disease are designed to decrease the toxicity of treatment without reducing the overall excellent results.

Staging

Once Hodgkin's disease is found, more tests will be done to determine whether the cancer has spread from where it started to other parts of the body. This is called staging. The plan of treatment depends on the staging.

Your doctor may determine the stage of the disease by examination, blood tests and x-rays. This is called clinical staging. In some cases, your doctor may need to do an operation (called a laparotomy) to determine the stage of the cancer. During this operation, the doctor cuts into your abdomen and carefully looks at the organs inside to see if they contain cancer. If a biopsy is done, this is called pathological staging. Pathological staging is usually done only when it is needed to help your doctor plan your treatment.

Bone Marrow Transplantation

Bone marrow transplantation is a newer type of treatment. Sometimes, Hodgkin's disease becomes resistant to treatment with radiation therapy or chemotherapy. Very high doses of chemotherapy may then be used to treat the cancer. Because the high doses of chemotherapy can destroy bone marrow, marrow is taken from your bones before treatment. The marrow is then frozen and you are given high doses of chemotherapy with or without radiation therapy to treat the cancer. The marrow which was previously taken is then thawed and given to you through a needle in a vein to replace the marrow that was destroyed. This type of transplant is called an autologous transplant. If the marrow you are given is taken from another person, the transplant is called an allogeneic transplant. Cells for the transplant may be taken from your blood for a procedure called peripheral stem cell transplantation.

Recurrent Adult Hodgkin's Disease

Patients who experience a relapse after initial wide-field radiation therapy have a good prognosis. Combination chemotherapy results in 10-year disease-free and overall survival rates of 7 to 80 percent and 7 to 81 percent, respectively.

Patients who experience a relapse after initial combination chemotherapy, especially if the recurrence occurs within the first 12 months of treatment, have a poor prognosis, although some improvement can occur with conventional chemotherapy. Prognosis is determined more by the duration of the first remission than by the specific induction or salvage combination chemotherapy regimen.

Patients whose initial remission was longer than one year (late relapse) have long-term survivals with salvage chemotherapy of 22 to 4 percent. Patients whose initial remission was shorter than one year (early relapse) do much worse and have long-term survivals of 11 percent.

For the small subgroup of patients with only limited nodal recurrence following initial chemotherapy, radiation therapy with or without additional chemotherapy may provide long-term survival for about 50 percent of all patients. The best results appear in patients who are aggressively re-staged and re-treated with wide-field (subtotal nodal irradiation or total nodal irradiation) high-dose radiation therapy, or more limited (mantle) irradiation and combination chemotherapy. Initial Stage IV disease may be a contraindication for this treatment. If it is used, there should be no evidence for disseminated disease at the time of nodal relapse.

For the remainder of patients, trials using high-dose chemotherapy and autologous bone marrow and/or peripheral stem cell rescue should be considered. These trials have resulted in three to four year, disease-free survival rates of 27 to 47 percent. Patients who are responsive to additional chemotherapy may have a better prognosis.

Questions to Ask Your Doctor

Do the symptoms indicate a diagnosis of Hodgkin's disease?

Are further tests necessary?

What is the staging of the disease?

Has it spread beyond the original site?

What treatment do you recommend?

Will chemotherapy or radiation therapy be needed?

Would bone marrow transplantation help if chemotherapy is needed?