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23 March 2011

Hodgkin's disease

Lymphomas are cancers that develop in the lymph system, part of the body's immune system. Lymphomas are divided into two general types: Hodgkin's disease and non-Hodgkin's

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Summary

  • Lymphomas are cancers that develop in the lymphatic system, part of the body's immune system.
  • Lymphomas are divided into two general types: Hodgkin's lymphomas and non-Hodgkin's lymphomas.
  • Adult Hodgkin's disease most commonly affects young adults and people older than 55.
  • It may also be found in patients with HIV/Aids, who are more prone to lymphoproliferative disorders.
  • Typical symptoms: painless swelling of the lymph nodes in the neck, underarm or groin; fever; tiredness; weight loss and itchy skin.

Description

Lymphomas are cancers that develop in the lymphatic system, which is part of the body's immune system. Lymphomas are divided into two general types: Hodgkin's lymphomas and non-Hodgkin's lymphomas. The diagnosis of different lymphomas is based on:

a) Their appearance under a microscope (histology), and
b) The markers on the lymphoma cells’surface (immunophenotyping/immunohistochemistry).

Adult Hodgkin's disease most commonly affects young adults and people older than 55. It may also be found in patients with HIV/Aids; these patients require special treatment. Hodgkin's disease can also occur in children, where it is treated differently from the disease in adults.

Symptoms

A doctor should be seen if any of the following symptoms persist for longer than two weeks: painless swelling of the lymph nodes in the neck, underarm, or groin; fever; night sweats; tiredness; weight loss without dieting; itchy skin.

If there are symptoms, a doctor will carefully check for swelling or lumps in the neck, underarms and groin.

Diagnosis and staging

If abnormal lymph nodes are present, a lymph node has to be removed surgically to confirm the diagnosis. The lymph node is sent to a laboratory, where it is evaluated by a specialist pathologist and the diagnosis and subtype of lymphoma are determined. Several other tests are then indicated to stage the disease. They include CT scan of chest, abdomen and pelvis, bone marrow aspirate, trephine biopsy and several blood tests.

The chance of recovery (prognosis) and choice of treatment depend on the stage of the lymphoma (whether it is just in one area, or has spread throughout the body); the size of the swollen areas; the results of the blood tests; the type of symptoms; and the patient's age, sex and general physical condition.

Treatment

Treatment can be offered to all patients with adult Hodgkin's disease. Two types of treatment are used:

  • Radiation therapy (using high-dose X-rays or other high-energy rays to kill cancer cells and shrink tumours)
  • Chemotherapy (using drugs to kill cancer cells and shrink tumours)

The mainstay of treatment is chemotherapy-based, but combination therapy (chemo- and radiation therapy) is also frequently used. Use of radiation therapy only is restricted for use in patients with very early-stage disease.

Radiation therapy is the use of high-energy X-rays to kill cancer cells and shrink tumours. Radiation for 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 radiation therapy to a mantle field.

Radiation therapy given to the mantle field as well as to the lymph nodes in the upper abdomen, the spleen and the lymph nodes in the pelvis is called total nodal irradiation. Radiation therapy may be used alone or in addition to chemotherapy.

Chemotherapy is the use of drugs to kill cancer cells and shrink tumours. 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 bloodstream, travel through the body, and can kill cancer cells throughout the body.

Bone marrow transplantation (peripheral blood stem cell transplantation) is a treatment option for appropriate selected patients. Sometimes Hodgkin's disease becomes resistant or responds very slowly to radiation therapy or chemotherapy. Very high chemotherapy doses are then used. Because these high doses destroy the patient's own bone marrow, an adequate number of peripheral blood stem cells (precursor cells present in the bone marrow in large numbers but also circulating in lesser numbers in the peripheral blood) are taken from the patient before starting with high-dose treatment. The stem cells are then frozen, and the patient is given high-dose chemotherapy with or without radiation therapy to treat the cancer. The stem cells are then thawed and given back to the patient intravenously. They find their way back to the patient's bone marrow which they recolonise and start to replicate. By doing so they replace the bone marrow that was destroyed.

This type of transplant is called an autologous transplant. If the stem cells are taken from another person (almost always a compatible brother or sister), the transplant is called an allogeneic transplant.

Treatment by stage

Patients may be immunized with influenza, pneumonia and meningitis vaccines before treatment and every few years after treatment, to guard against infection. Treatment of adult Hodgkin's disease depends on the type and stage of the disease, size of the lymph node masses, the patient's age, pregnancy status, past surgery to determine the stage of the disease, certain laboratory markers, clinical symptoms and general health.

Standard treatment may be considered based on its effectiveness in past studies, or participation in a clinical trial may be considered. Not all patients are cured with standard therapy and some standard treatments may have more side effects than are desired.

Within five to 15 years after treatment, some patients develop another form of cancer as a result of their treatment; therefore they should visit a doctor regularly to be checked.

Stage I Adult Hodgkin's Disease
The disease involves only one group of nodes. Treatment depends on whether the patient has stage IA or stage IB disease and where the cancer is found.

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

  • Combination chemotherapy with radiation therapy
  • Radiation therapy only, given to a mantle field
  • Clinical trials of combination chemotherapy only

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

  • Radiation therapy to a mantle field plus chemotherapy
  • Radiation therapy to a mantle field and to the lymph nodes in the upper abdomen

If the cancer is below the diaphragm, treatment may be one of the following:

  • Radiation therapy
  • Combination chemotherapy with radiation therapy
  • Clinical trials of chemotherapy alone

Stage IB disease: Treatment may be one of the following for patients with "B" symptoms:

  • Combination chemotherapy with radiation therapy
  • Clinical trials of chemotherapy alone

B-symptoms are: The presence of fever above 38°C or night sweats in the last month and/or unexplained loss of 10% or more body weight in the preceding six months.

Stage II Adult Hodgkin's Disease
More than one group of lymph nodes are involved, on one side of the diaphragm (either upper or lower part of the body, but not both). Treatment depends on whether the patient has stage IIA or stage IIB disease and where the cancer is found.

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

  • Combination chemotherapy and radiation therapy
  • Radiation therapy to a mantle field and to the lymph nodes in the upper abdomen
  • Radiation therapy to a mantle field only, after surgery to determine the stage of the tumour. This treatment option is rarely used these days
  • Clinical trials of combination chemotherapy alone

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

  • Chemotherapy plus radiation therapy to a mantle field
  • Chemotherapy alone

Stage IIB: Treatment may be one of the following for patients with "B" symptoms:

  • Combination chemotherapy with or without radiation therapy to places of bulky tumour
  • Clinical trials of chemotherapy alone

Stage III Adult Hodgkin's Disease
Disease involving lymph nodes on both sides of the diaphragm (upper and lower body), but not the bone marrow.

Stage III A and III B: If the cancer does not involve a large part of the chest, treatment may be one of the following:

  • Combination chemotherapy alone
  • Combination chemotherapy followed by radiation therapy to any residual tumour that remains after the chemotherapy has been completed
  • Total or subtotal nodal irradiation only
  • A clinical trial of chemotherapy

If the cancer involves a large part of the chest, treatment should be:
Combination chemotherapy followed by radiation therapy to the initial site of bulky disease.

Stage IV Adult Hodgkin's Disease
This is widespread disease frequently involving the bone marrow. Treatment may be one of the following:

  • Combination chemotherapy
  • Combination chemotherapy and radiation therapy
  • Clinical trials of chemotherapy with bone marrow transplantation

Recurrent Adult Hodgkin's Disease
The salvage treatment in this setting depends on:

  • Place of recurrence
  • Treatment received before
  • General condition of the patient

If the treatment received before was radiation therapy without chemotherapy, chemotherapy may be given. If the treatment received before was chemotherapy without radiation therapy and the cancer comes back only in the lymph nodes, radiation therapy to the lymph nodes with or without more chemotherapy may be given. If the disease comes back in more than one area, more chemotherapy may be given or a clinical trial of high doses of chemotherapy with bone marrow or peripheral stem cell transplantation may be presented as an option.

Previously reviewed by Dr Adrian Schmidt, MBChB (Pret), MMed Internal Medicine (Stell)

Reviewed by Dr David Eedes, Oncologist, March 2011

 
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