AIDS Related Lymphomas

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What is the lymphatic system?
What is AIDS?
What are Lymphomas? How are they classified?
How does AIDS related Lymphoma present?
What are staging and grading?
How are Lymphomas treated in AIDS patients?
What are the side effects of treatment for AIDS-related Lymphoma?
What is the importance of follow up?
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What is the lymphatic system?
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The lymphatic system is a network of thin transparent tubes that, just like blood vessels, permeate to all parts of the body. They are carriers of a colorless watery fluid called lymph which contains white blood cells called lymphocytes. Along the network of vessels are multiple stations or junctions which are small, bean-shaped organs called lymph nodes.

Lymph nodes are arranged discretely as well as in major clusters located in the pelvis, in the neck, along the back of the abdomen, and under the armpits. The lymph nodes are basic stations for making and storing infection-fighting cells. These are part of the reticuloendothelial system which also contains the spleen, which is an organ in the upper abdomen that makes lymphocytes and filters old blood cells from the blood, the thymus, which is a small organ beneath the breast bone and the tonsils which are multiple sets of lymphoid tissue located on the back of the throat and along the side walls of the oral cavity.

Besides these members of the reticuloendothelial system, lymphatic tissue is almost omnipresent in various organs of the body, especially in the digestive system where it is known as GALT which stands for gut-associated lymphatic tissue.

Lymphatic System
Lymphatic System
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What is AIDS?
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The Lymphatic in the Legs (Front and back views).
The Lymphatic in the Legs (Front and back views).

AIDS or the acquired immunodeficiency syndrome is caused by the human immunodeficiency virus or HIV, which has a predilection to attack and weaken the body's defence and resistance mechanisms against infection and diseases. Various conditions can be produced in AIDS besides infections. One of the conditions is Lymphoma.
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What are Lymphomas? How are they classified?
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Lymphomas are cancers which involve the lymphatic system; the part of the body's immune system.

They also have the predilection to spread to almost any organ or tissue, including the bone marrow which is the spongy tissue inside the large bones of the body that is the principle maker of blood cells, the liver or the spleen.

Lymphomas are classically divided into two general categories: Hodgkin's disease and non-Hodgkin's Lymphoma. The cancer cells in Hodgkin's disease are identified because of their certain look, which is seen under the microscope. All other varieties of Lymphomas which do not have the cell characteristics of Hodgkin's disease cell are classified into non-Hodgkin's Lymphomas.

Both Hodgkin's disease and non-Hodgkin's Lymphomas may occur in an AIDS patient. The difference between a normal patient and AIDS patients as far as Lymphomas are concerned is that usually in AIDS patients, the Lymphomas are often intermediate or high-grade variety, rather than the indolent variety. The Lymphomas can occur either in adults or in children.

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How does AIDS related Lymphoma present?
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AIDS related Lymphoma is suspected when the doctor finds the following symptomatology persistent in a person for greater than 2 weeks, and progressing at a rapid rate:
Fever.
Night sweats.
Extreme tiredness without cause.
Weight loss without dieting or known cause.
Itchy skin.
Painless swelling in groups of lymph nodes in the neck, groin or underarm.
If there are symptoms such as these, a doctor first carefully evaluates all the known areas of the lymph node stations. If the lymph nodes reveal any abnormalities, the doctor needs to perform a biopsy which is the removal of a small piece of tissue from the lymphatic stations to get it examined by the pathologist under the microscope for detection of cancer cells.

The doctor also may order a series of investigations including a CT scan of the chest or abdomen, x-rays, or ultrasonographies and various blood studies.

The term immunohistochemistry may also pop up in the lexicon, which basically is a detailed study for Lymphoma patterns in the tissue which has been taken out from inside the patient.

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What are staging and grading?
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These are terms with which you must become familiar, if you are dealing with any form of cancer.

When we stage a cancer, we try to establish carefully degree of spread of the cancer and if indeed there is spread, to what extent and involving which organs.

The grading of a cancer is a microscopic issue, in which the pathologist lets you know how aggressive this particular type of cancer is; well differentiated means less aggressive, moderately differentiated intermediately aggressive and poorly differentiated more aggressive.

Both grading and staging help your clinician to establish the prognosis or how serious your disease is and what is going to be the likely outcome.

Staging may require additional imaging tests such as CT Scan, MRI, Sonography of the abdomen, IVU, Bone scan or chest X-ray.

For AIDS-related Lymphomas, a slightly different grouping is used, which is as follows:

Systemic or peripheral Lymphoma.
Lymphoma, which starts in the lymph nodes or other organs of the lymph system. The Lymphoma has spread from its origin to all parts of the body including the brain or bone marrow.

Primary central nervous system Lymphoma.
Lymphoma is originated in the central nervous tissues, i.e., the brain or the spinal cord and from there it is either still located in the CNS or it has spread to other parts of the body after originating from the CNS. There is a slight difference here. Those Lymphomas which have originated elsewhere and have spread to the CNS are not included in primary CNS Lymphomas.

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How are Lymphomas treated in AIDS patients?
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The usual parameters for treatment in patients with AIDS is distorted as compared to the general population as the response of patients with AIDS-related Lymphomas is quite different from those who do not have AIDS.

AIDS-related Lymphomas are faster growing and spread rapidly outside the nodes and to other parts of the body. Because treatments for Lymphomas weaken the immune systems even further, it is important that AIDS-related Lymphoma patients are treated with lower doses of drugs than patients who do not have AIDS. This is obviously a compromise, but there are no alternatives to this route being taken.

A lot depends on the patient's constitution, the grade of his disease and its staging. Patients are usually treated by a team of specialists which utilizes a multipronged approach. This includes a medical oncologist,an oncosurgeon and a radiotherapist. The plan of treatment is tailored to fit a patient's requirements.

Treatment strategies

Treatment in AIDS-related Lymphomas is a difficult proposition because of the problems caused by HIV infections, which are recurrent and continuously weaken and destroy the immune system of the person. Drug dosages also have to be compromised upon and lower doses have to be used than those patients who do not have AIDS.

The bastions of treatment in AIDS-related Lymphomas are chemotherapy and radiotherapy.

The best attempt to control cancer cells circulating in the body is by using systemic therapy (therapy which affects the entire systems of the body) which is in the form of injections or oral drugs. This form of treatment is called chemotherapy.

This form of treatment is given in a cyclical manner (each set of drugs is repeated usually after every 3-4 weeks.)

Chemotherapy can also be used in combination with surgery, radiotherapy or both, either before or after.

Chemotherapy in Lymphoma is quite aggressive and uses multiple drugs and this is called combination chemotherapy.

Inside the layers covering the spinal cord is the fluid which is known as cerebrospinal fluid. Chemotherapy may have to be given intrathecally as well, that is by injecting into the cerebrospinal fluid.

These treatments are often combined with the use of drugs which are used to kill the AIDS virus (antiviral treatment).

AIDS-related primary or systemic Lymphoma.

Treatment may follow one of the following options:
Systemic chemotherapy in standard doses plus intrathecal chemotherapy.
Low-dose systemic chemotherapy plus intrathecal chemotherapy.
Newer modalities of chemotherapies or new ways of giving chemotherapy.
All these or none of these may be combined along with broad-spectrum antiviral therapy or specific antiviral therapy directed towards the AIDS virus.

AIDS-related primary CNS Lymphomas.

Treatment is usually cranial irradiation.
Other therapies may be combined on the basis of clinical trials which are proceeding worldwide.

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What are the side effects of treatment for AIDS-related Lymphoma?
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Unfortunately treatment for cancer does cause damage to the healthy tissues.

Please ask the clinician about any possible side effects before any form of treatment commences.

The effects of radiation depend upon the dose of radiation, the size of the area radiated, and the number and size of each fraction.

The commonest side effect is extreme fatigue. Although bed rest is good, most radiotherapists advise the patient to be as mobile as possible.

Another common problem is that of radiation dermatitis in which the skin covering the radiated area becomes red, dry, itchy, and may show signs of scaling off. This will slowly settle down after radiation ceases, but there may be a permanent 'bronzing' of the skin. It is important to note here that this skin problem is associated only with teletherapy or external radiation therapy.

Radiation also may cause nausea and vomiting, diarrhea, urinary discomfort.

There may also be a fall in the white blood cells, which are needed by humans to fight infection.

Usually the radiotherapist can suggest the drugs and diet necessary to alleviate such problems.

The various drugs in chemotherapy cause a varied spectrum of side effects. The severity and variability of these symptoms are very individualistic, changing from patient to patient.

Some of the common side effects are:
Skin rashes.
Loss of hair.
Diarrhea.
Vomiting.
Tingling and numbness in the fingers and toes.
Hearing loss.
Most are temporary and recede after therapy is over. Hair growth gradually starts after cessation of chemotherapy.

The medical oncologist will usually be able to reduce the severity and spectrum of these side effects.

The psychology of the patient is very important during therapy. Better psychologically prepared the patient is, better are his chances of having decreased side effects. The patient should drink large quantities of water while the therapy is in progress, and preferably avoid uncooked or raw food. Water should be filtered or boiled. Any relative or person who has any sort of infection such as common cold should be asked to desist from coming close to the patient.

AIDS patients are particularly vulnerable to all side effects, in a greater degree of exaggeration, because of their decreased resistance to any form of foreign assault, either infective, carcinogenic or otherwise.

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What is the importance of follow up?
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The importance of follow-up cannot be overemphasized. It is a fatal fallacy to assume on the patient's part that once the primary or the first phase of treatment is over, then he or she is cured. It must be stressed here that cancer has a notorious proclivity of coming back again and again. The only way to fight this is to pick up any problem when it is still early, so that it can be nipped in the bud. And the only way to do this is to follow up when the doctor calls you for the next visit. Each such visit will include a detailed history, a physical examination (including an internal i.e. a rectal or vaginal examination), a chest x-ray, an ultrasonography or a CT scan, and various other blood studies as indicated.

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