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LYMPHOMA FACT SHEET


Overview

Lymphomas are cancers that develop from lymphocytes – the cells of the lymphatic system. There are many different types of lymphomas which are divided into two main groups - Hodgkin's and non-Hodgkin's lymphomas.

Lymphoid tissue is found all over the body, including the bone marrow, tonsils and spleen. There are also a large number of small patches of lymphoid tissue found in the gut, tonsils and other sites around the body. Lymphocytes initially develop in the bone marrow from stem cells and then differentiate in either the thymus (T-cells) or the bone marrow (B-cells). T-cells identify and attack foreign cells, such as bacteria. B-cells make antibodies.

Lymphomas can also start in the brain - particularly in people who have had an organ transplant or have AIDS, because their immune systems are suppressed. Lymphomas in the brain and spinal cord are not the same as lymphomas found in the rest of the body and tend to be more difficult to treat.

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Key Figures

Figures given for incidence and mortality of lymphoma are available for the EEA and EU25 and for non-Hodgkin's lymphomas only. As a guide to total numbers, non-Hodgkin's lymphomas are roughly 4 times more common than Hodgkin's lymphomas.

Total number of new cancer cases in Europe (2006)      3.2 million
Total number of cancer deaths in Europe (2006) 1.7 million
Number of new non-Hodgkin's lymphoma cases (2006)      74,800 (EEA)
(40,600 men)
(34,200 women)
72,800 (EU25)
(39,600 men)
(33,200 women)
Number of non-Hodgkin's lymphoma deaths (2006) 33,900 (EEA)
(17,600 men)
(16,300 women)
33,000 (EU25)
(17,100 men)
(15,900 women)

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Cancer Types

There are two main groups of lymphoma:

The main T-cell lymphomas are:

Another way of classifying non-Hodgkin's lymphomas is to categorise them as indolent or aggressive lymphomas.

  • Lymphomas that grow slowly are called indolent or low-grade lymphomas. Some indolent lymphomas may need some treatment or in some cases just careful surveillance.
  • Lymphomas that tend to grow quickly are called aggressive, intermediate or high-grade lymphomas. Aggressive lymphomas require urgent treatment to prevent them from becoming life-threatening.

Over time, an indolent lymphoma can sometimes change into an aggressive lymphoma.

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Causes of Lymphoma

  • Lymphoma is a disease of both children and adults.
  • The majority of people with lymphoma are over the age of 60.
  • Immune system diseases such as HIV infection and AIDS weaken the immune system and make the body more susceptible to some forms of lymphoma.
  • Lymphomas are not hereditary.

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Prevention of Lymphoma

There are no practical steps that can be taken to reduce the chances of developing lymphoma.

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Common Symptoms

The classic symptoms of lymphoma are:

  • Swelling of the lymph nodes - usually most noticeable in the neck, armpit or groin. The swelling is usually painless though swollen glands can sometimes be sore. Painful swollen glands are more likely to be due to infections.
  • Pain in the lymph nodes after drinking alcohol is a peculiar symptom that is more common in Hodgkin’s lymphoma.
  • Fever and sweating are common general symptoms especially with infections. However, in lymphoma this fever and sweating typically occurs at night. They are more commonly associated with Hodgkin’s lymphoma.
  • Persistent itching – although this is another symptom that occurs in a wide range of conditions.
  • Abdominal swelling and pain. In lymphoma, this is due to an enlarged spleen and/or lymph glands.
  • Weight loss, poor appetite and tiredness are all very general symptoms which can be due to a number of causes, but also commonly occur with lymphomas.

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Diagnostic Tests

Diagnosis is usually confirmed by a biopsy of the enlarged lymph nodes.

If a lymph node biopsy confirms the diagnosis of lymphoma, further tests are required to determine the extent of the spread of the cancer. These tests may include: a chest X-ray, a computerised tomography (CT) scan, and/or a magnetic resonance imaging (MRI) scan, and a bone marrow biopsy.

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Treatment

Different treatments will be recommended according to:

  • the type of lymphoma
  • its stage

Available treatments include:

Chemotherapy

Many different combinations of drugs are used to treat lymphomas (including doxorubicin, vinblastine, vincristine, bleomycin, etoposide and steroids). However, for B-cell lymphomas, rituximab is the first line of treatment.

Chemotherapy and radiotherapy can be used in combination (chemoradiation) to increase the effectiveness of treatment.

Radiotherapy

External beam radiotherapy is normally given as a series of short regular treatments in a radiotherapy department (each treatment may be referred to as a fraction).

Radiotherapy can be combined with chemotherapy in the treatment of lymphoma.

Monoclonal Antibody Therapy

Monoclonal antibodies are a form of immunotherapy which are now commonly used in conjunction with chemotherapy in treating B-cell lymphomas.

Bone Marrow Transplant

Autologous bone marrow transplants are sometimes used in the treatment of lymphoma so that high-dose chemotherapy can be given to destroy lymphoma cells.

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Treatments for Specific Lymphomas

Hodgkin's lymphoma

Hodgkin's lymphoma is treated with chemotherapy and radiation.

Radiotherapy is given to the region of affected lymph nodes as a series of short regular treatments in a radiotherapy department (each treatment may be referred to as a fraction).  Treatment is given over several weeks to minimise side effects.

Chemotherapy uses a combination of two or more drugs which are given in cycles. Chemotherapy regimens are identified by their initials. Specific drugs used include doxorubicin, bleomycin, vinblastine and dacarbazine but other agents including prednisolone are also used.

New medications and combinations are being developed to increase effectiveness and minimise side effects and long-term complications.

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Non-Hodgkin’s Lymphomas

Follicular lymphoma is often slowly growing and with close monitoring treatment can be delayed until the lymphoma is causing symptoms. Where the lymphoma is localised to a single lymph node group, it can be treated with radiotherapy to the affected area.

When follicular lymphoma does need treatment, chemotherapy using either a single drug (often fludarabine or chlorambucil) or a combination of drugs (such as cyclophosphamide, vincristine and prednisone) is used. However, there are many alternative effective regimens.

Anaplastic large cell lymphoma is a fast growing lymphoma that mainly affects lymph nodes and is treated with a variety of chemotherapy regimens (sometimes with radiotherapy). Response to treatment is generally good. If the lymphoma returns after initial treatment, an autologous stem cell transplant is an option.

Peripheral T-cell lymphomas are generally treated with chemotherapy. In the early stages of the disease, radiation therapy may be an option.

Primary central nervous system lymphoma. As the name implies, this lymphoma starts in the brain or spinal cord (see also section on Brain Tumours). The symptoms are like those of other brain tumours and the lymphoma often develops in older people or those with immune system problems caused by AIDS or drugs given to prevent rejection of transplanted organs. Treatment is with chemotherapy and/or radiotherapy.

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Prognosis

Many factors affect prognosis, including the stage of the lymphoma, the patient’s age, general health and their individual response to treatment (which will vary). For this reason statistics can only be considered as a general guide as they are produced by grouping together patients in whom these factors are similar but not identical. These figures are historical, often covering a 10 or 20 year period, and so do not reflect improvements in survival through more modern treatment.

Survival rates indicate the percentage of people who survive the disease for a specific period of time after their diagnosis – usually 5 or 10 years.

In Hodgkin’s Lymphoma overall cure rates (including all stages and all patients) are between 75% and 80%. For young patients with early stage disease, almost all patients can expect to be cured.

Patients with indolent Non-Hodgkin's lymphoma types have a median survival of 10 years. These types of tumours are usually not curable when advanced.

Patients with aggressive lymphomas are more responsive to chemotherapy and have a median survival time of between 2-5 years (intermediate grade) and less than 2 years (high-grade). Between 30% and 60% can be cured.

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Key Trends

The main recent development has been the introduction of monoclonal antibodies and radioimmunoconjugates in the treatment of B-cell lymphomas.

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Important Research Areas

  • Further use of various monoclonal antibodies in the treatment of lymphomas.
  • An increasing number of cytogenetic and molecular abnormalities are now being identified in various subtypes of Non-Hodgkin’s Lymphoma. Some of these abnormalities may provide prognostic information and be useful as tumour markers to check for the presence of residual disease after treatment.
  • Defining the place of radiotherapy in the treatment of lymphoma.

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