Cancer Support Group

Friday, Sep 22nd

Last update:06:42:40 AM GMT

Lymphomas

Overview of Lymphomas
Lymphomas are cancers of the lymphoid cells which are part of the lymphatic system. The lymphatic system is a series of fine vessels similar to blood vessels. It drains fluid away from the tissues and returns it to the bloodstream. Throughout the system there are small organs called lymph nodes (sometimes called lymph glands). There are clusters of lymph nodes in the neck, armpits and groins and deeper in the chest, abdomen and pelvis.

The spleen (an organ in the upper abdomen), the thymus (an organ under the breastbone) and the tonsils and adenoids (in the throat) are also part of the lymphatic system. The lymph nodes often become swollen in response to an infection, for example the swollen glands associated with a throat infection. Lymph nodes are packed with lymphocytes which are a type of white blood cell. These are grouped within the lymph node in a very specific way according to their function. One classification of lymphoma is based on which part of the node is involved and how like a normal lymph node the structure of the affected node appears.

Signs and Symptoms
In both forms of lymphoma the first sign noticed by the patient is usually firm painless swelling of the lymph nodes (swollen glands). In contrast the swollen nodes caused by infection are usually tender. In both forms general symptoms may develop as the disease progresses. There are two peak ages of onset of Hodgkin’s Disease, one between 15 and 30 years and the other over the age of 50 years. This is called as bimodal distribution of the disease. Non–Hodgkin’s lymphoma is mainly a disease of older people.

The condition usually starts as painless enlargement of lymph nodes. Common sites where the nodes are enlarged are the neck, axilla, groin. The liver, spleen may also enlarge giving a sense of abdominal discomfort to the patient. About a quarter of patients will have fever, night sweats, unexplained weight loss, itching. If the lymph nodes in the chest are enlarged they have a cough and chest pain. Non–Hodgkin’s disease also presents with painless lymph node enlargement. The nodes in the abdomen are more likely to be involved as compared to Hodgkin’s disease. Also symptoms such as fever, night sweats, itching is less common in Non–Hodgkin’s lymphoma.

Diagnosis and Staging
In all cases of lymphoma it is necessary to take a sample of the affected node(s) to confirm the diagnosis and to establish the exact type of the disease. This is called a lymph node biopsy and allows the node to be examined in the laboratory. If the affected node(s) are all inside the body then the biopsy may be done using a fine needle under ultrasound or CT control. Usually the whole of the affected node(s) is removed. This is called an excision biopsy. The appearance of the node under the microscope will determine whether it is classified as Hodgkin’s Disease or Non–Hodgkin’s Lymphoma.

Hodgkin’s Disease is classified into four types depending on the appearance under the microscope. Non–Hodgkin’s lymphoma is further classified by special tests which show which types of lymphocytes are involved. This classification of the lymphoma is important because it affects the treatment choice.

Staging
In both forms of lymphoma it is essential to discover how widely the disease has spread because many of the lymph nodes are inside the body and cannot be seen or felt directly. The methods used are the same for Hodgkin’s Disease and Non–Hodgkin’s lymphoma. This is called staging and is very important because it is a key factor in deciding what treatment to give. Usually staging involves blood tests, a biopsy of the bone marrow and special X–rays.

It is very important to know whether or not lymphoma cells are present in the bone marrow and so it is normal to take a small sample of bone marrow. This is done by anaesthetizing the skin and underlying bone on the back of the hip bone. A small needle is then inserted into the bone cavity and a little of the marrow is removed by sucking it into a syringe. A thin core of bone and bone marrow is also removed. This procedure should not take long although some discomfort may occur as the marrow is actually removed.

A CT (Computed Tomography) scan which may be called a body scan or a CAT scan is always conducted. Another method which is becoming more common and may be used for staging lymphoma is called an MRI (Magnetic Resonance Imaging) scan.
The staging system is the same in both types of lymphoma.

Types of Lymphoma
There are many different types of lymphoma but historically two broad groups have been recognized. One group is called Hodgkin’s Disease and the other Non–Hodgkin’s Lymphoma. The difference is based on the difference in the age group at presentation, the pattern of distribution of the lymph nodes and the presence of a large abnormal cell (called the Reed–Sternberg cell) which is present in Hodgkin’s but not in Non–Hodgkin’s disease. Treatments of Hodgkin’s Disease and Non–Hodgkin’s lymphoma are different and it is important to be sure of the diagnosis.

Radiotherapy
Common side effects of radiotherapy include general tiredness and mild nausea, although the latter can usually be controlled by simple medication. Radiotherapy can cause hair loss, but this is confined to the area being treated. In recent years it has been recognized that there are some uncommon, but important, long–term side effects of radiotherapy. Patients who have received radiotherapy to the center of the chest may suffer from heart disorders later in life. This is due to the effects of radiotherapy on the blood supply to the heart. There is also some evidence that radiotherapy may cause some other types of cancer, occurring many years after the treatment.

Although these conditions may sound alarming it is important to emphasize that they are very uncommon. It is important to stress that with modern treatment the dose and extent of radiotherapy given is lower than in the past and it is likely that these long–term complications will be very rare.

Chemotherapy
Common short–term side effects of chemotherapy include nausea and vomiting, tiredness, hair loss and soreness of the mouth. Nausea and vomiting are usually problems in the first 24 to 48 hours after chemotherapy is given. Various anti–sickness treatments are available including injections which can be given at the time of chemotherapy, and tablets which can be used later. Modern anti–sickness treatments are very effective and many new patients have few, if any, severe problems of this type.

Loss of hair is another common problem with chemotherapy. It is temporary and hair will grow back once chemotherapy is complete. Mouth soreness is usually treated with various mouthwashes. Individual hospitals provide their own guide–lines for mouth care. Other important side effects do occur. The more intensive chemotherapy treatments may cause a temporary severe lowering of the the white cell and platelet counts. Antibiotics to prevent infection and platelet transfusions to prevent bleeding may be given until the patient’s white cell and platelet production recover. New drugs called growth factors, which are artificial versions of natural body chemicals, may be used to speed up the recovery of blood cell production.

In the long term chemotherapy can affect the blood producing system and lead to a condition called Myelodysplasia. With modern treatments this is very rare. Some of the chemotherapy drugs used can also affect the function of the heart. When these forms of chemotherapy are used it is important that the dose given does not exceed a critical level. Below this level heart problems are very rare. As with radiotherapy, chemotherapy is also associated with some long–term side effects. Of these, the most important is the effect on fertility. Until recently, chemotherapy for Hodgkin’s Disease almost always resulted in infertility in men. In recent years new types of chemotherapy have been introduced and the number of men who are fertile after treatment is increasing. It is not yet possible, however, to say what proportion of men will have normal fertility after modern chemotherapy.

Most men will have reduced sperm counts and thus reduced fertility during chemotherapy. Despite this, it is still essential to continue with some form of contraception. Most hospitals recommend that patients who have received chemotherapy should not plan to have children until about 1 year has passed from the end of treatment. After this period of time there is no evidence to suggest that the likelihood of abnormality in the baby is increased.

Reduced fertility is also a problem in women treated with chemotherapy for Hodgkin’s disease, although, as with men, it is less significant with modern chemotherapy. In the past women patients often experienced an early menopause, but this is less likely with new treatments. Again, contraception must be continued throughout treatment. It is important to recognize the possibility of becoming pregnant during chemotherapy which may harm a developing baby.

Stem Cell Transplants
It is a novel treatment and is being evaluated in patients who relapse after receiving chemotherapy. It is also now being used much more frequently following “Normal” chemotherapy. However it is not yet proven to be of value as part of first line treatment and is currently being assessed as part of clinical trials.

Follow Up
Patients who have completed treatment for Hodgkin’s Disease or Non–Hodgkin’s lymphoma are followed for a period of many years.

Typically patients are seen at one to two monthly intervals for the first few follow up visits. If there are no significant problems the intervals between visits will become longer. During this period patients are carefully checked for symptoms which might suggest recurrence of the disease. Any tests which were abnormal at the beginning of treatment are repeated to monitor the response. This may involve blood tests and scans or X–rays. If the bone marrow biopsy was abnormal this will usually be repeated. The number of tests required will be reduced as more time passes. It is unrealistic to expect the doctor to diagnose early relapse if the patient is seen only rarely. It is important that patients understand the symptoms of relapse and that the doctor is consulted urgently if any of these are noticed. Using this approach the most appropriate investigation is often a CT scan at the earliest opportunity. Symptoms which may warn of a relapse are new lymph node swellings, fevers or sweats, increasing weight loss, infections and temperatures.

Hodgkin’s Lymphoma
Causes of Hodgkin’s Disease
There is evidence that at least some cases of Hodgkin’s Disease are caused by a specific virus infection, the Epstein–Barr virus. Epstein Barr virus infection is very common and the reason why a small number of people with the infection go on to develop Hodgkin’s Disease is not known. There is speculation that other viruses may also be associated with Hodgkin’s Disease.

Treatment for Hodgkin’s Disease
Treatment for Hodgkin’s Disease typically involves chemotherapy, radiotherapy or a combination of both. The treatment will depend on the exact sub–type and the stage of the disease i.e. how far it has spread.

Treatment depends on the exact stage, the sub–type, the size of the glands and the results of blood tests. The major decision is balancing the chances of a complete cure against the likely side–effects of treatment. Most patients with early stage of disease are curable. If only a single node is involved local radiotherapy alone may be given.

For most other patients with early stage disease, treatment involves more extensive radiotherapy, chemotherapy or both. If the disease is in the upper half of the body, radiotherapy to the neck, under the arms and the central part of the chest may be given. This is known as upper mantle or extended mantle radiotherapy.

For patients with disease in only the lower half of the body radiotherapy is sometimes given to the groins, the pelvis and the central part of the abdomen. This is known as inverted Y radiotherapy. If radiotherapy alone is used a proportion of patients are liable to have a relapse, that is the disease will return, and they will need further treatment. When this happens the majority of patients can be cured with Chemotherapy.

The risk of relapse can be greatly reduced if both treatments are combined at the outset. The disadvantage of doing this is that the patient has side–effects from the chemotherapy as well as radiotherapy. For this reason combined therapy is usually reserved for patients who are thought most likely to relapse if they have either radiotherapy or chemotherapy alone. Courses of chemotherapy are usually given first and the radiotherapy follows after 2–4 weeks. The radiotherapy is often less extensive and is given only to the affected areas.

Radiotherapy is usually given on an out–patient basis. The full treatment takes three to six weeks. There is a great deal of preparation needed to ensure that the radiotherapy is given accurately. The actual treatment takes a few minutes and is quite painless apart from some local irritation of the skin. Chemotherapy for early stage of Hodgkin’s Disease usually consists of a combination of drugs, some by injection and some by mouth. These are given on an outpatient basis, usually at three to four week intervals, for about four to six months.

The advanced stage of the disease affects multiple groups of nodes and causes symptoms in the patient. It also includes disease which has spread to lymph nodes on both sides of the diaphragm (the sheet of muscle between the chest and the abdomen), or to other organs like the liver and bone marrow. If the disease presents with very large nodes it is also treated as advanced even if only a few nodes are affected. This is especially the case if nodes in the center of the chest (the Mediastinum) are involved.

The main treatment for advanced Hodgkin’s disease is chemotherapy. The exact drugs and dose schedules may vary from one treatment center to another. Often the chemotherapy is given intravenously at three weekly intervals with a further injection one week after the first. Prednisolone tablets are a very important part of treatment and directly kill the lymphoma cells. One of the major problems with chemotherapy is that while it kills the lymphoma cells it also kills the normal white cells in the blood. As a result of this patients will be prone to infections. Such infections can progress rapidly and can be fatal. Because of this they must be treated with antibiotics at an early stage.

After three cycles of treatment it should be possible to notice a reduction in the size of any swollen nodes. If no swollen nodes can be felt the CT scan will be repeated to determine how well the internal nodes have responded to treatment. Provided there has been a good response the chemotherapy will be continued, often to a total of six to eight cycles. Following this, the CT scan will be repeated to ensure that the nodes have all gone. Radiotherapy may be used in advanced Hodgkin’s Disease, especially in patients with large nodes. It is usually given at the end of the course of chemotherapy.

Non Hodgkin’s Lymphoma
Causes of Non Hodgkin’s
The cause of Non–Hodgkin’s lymphoma is poorly understood. A small percentage of cases occur in people whose immune systems are not working properly e.g. after a transplant or because they have HIV infection. Some cases of lymphoma may occur after treatment for cancer with chemotherapy or radiotherapy. In the majority of cases no cause can be identified.

Treatment for Non Hodgkin’s
The treatment of Non–Hodgkin’s lymphoma depends on the grade and extent of the disease. The different types of Non–Hodgkin’s lymphoma are grouped into two patterns of clinical behavior depending on how quickly they grow. These patterns are indolent (slow growing) and aggressive. Treatment often involves chemotherapy or radiotherapy. Decisions about treatment are complex and must be made by specialists who are thoroughly familiar with these diseases and with the best forms of treatment.

Indolent
These are very slow growing lymphomas, the first sign of which is usually enlarged lymph nodes. These nodes are usually painless and have often been present for weeks, months or even years before the patient takes any action. They often “Come and go” over long periods of time without any treatment.

These types of lymphoma respond very well to treatment, but are not usually completely curable, unless they are diagnosed at a very early stage. Most patients will respond to treatment and remain in good health despite having the disease for very long periods of time.

If the disease is limited to one group of nodes, radiotherapy to the affected area may be used. Some people may be completely cured of the disease in this way. If the lymphoma is more widespread, the type of treatment depends upon the patient’s general condition. If the nodes are small and the patient has no symptoms no treatment is given and started only when it is evident that the lymphoma is progressing.

Many patients find it difficult to accept that they have a form of cancer and that the doctors are not planning any treatment straight away. However, this approach does not in any way reduce the patient’s chances of being alive and well years after the original presentation. When treatment is required it is usually because the affected lymph nodes are either large or causing pressure on other organs, or because the patient is unwell with symptoms due to the lymphoma. Chemotherapy is the recommended treatment. This is usually given in the form of tablets, which are taken intermittently over six to nine months. There are usually few side–effects from these tablets.

The chances of survival does not improve if very high–dose chemotherapy is given to patient with low grade lymphoma. However, in young patients experimental procedures such as autologous bone marrow or stem cell transplants are being evaluated.

High grade
This type of lymphoma is more rapidly growing than indolent lymphoma and always requires immediate treatment. The majority of patients, however, will require chemotherapy. This can take many different forms. Most commonly it is given as a series of injections and tablets at three week intervals for a total of six cycles. The whole course of treatment therefore takes about four months to complete.

Chemotherapy is usually given as an out–patient in a specialist clinic, although patients sometimes receive their first course of chemotherapy on the hospital ward if they are very unwell. Some patients with aggressive Non–Hodgkin’s lymphoma may receive radiotherapy at the end of chemotherapy particularly if there were areas with particularly large nodes at presentation. If the use of radiotherapy is considered advisable it will be discussed fully by the doctor.

Rare Types of Aggressive non–Hodgkin’s Lymphoma
There are two types of lymphoma which need special mention. They grow rapidly and may involve the brain. Treatment of the brain is required with radiotherapy or with chemotherapy given into the fluid surrounding the brain.

The first of the two types is called Lymphoblastic Lymphoma. This behaves identically to Acute Lymphoblastic Leukemia and should be treated as such. Lymphoblastic lymphoma has more of the disease as nodal lumps and does not affect the bone marrow as extensively as Lymphoblastic Leukemia.
The second type is called Burkitt’s Lymphoma.

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