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Learn About Non-Hodgkin's Lymphoma©
Written by Cancer Center Staff

Source: Cancer Resource Room

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What is Non-Hodgkin’s Lymphoma?
Lymphomas are cancers of the cells and tissues of the body's defense system. There are two parts to the defense (immune) system, one is a group of cells called white blood cells, and the other is made up of lymph tissues. Blood cells are made in the bone marrow, located in the center of bones. Most of the cells made in the marrow are red blood cells that carry oxygen to all cells of the body. The others are white blood cells that protect the body from unwanted or dangerous cells and wastes. White blood cells (WBCs) circulate with the blood and lymph.

Lymph is a fluid that collects outside cells and is made up of excess water, proteins, cell debris, and most importantly, White blood cells (WBCs).

Lymphatic System, Massachusetts General Hospital, Boston, MAThe lymph is filtered through several sites of lymph tissue throughout the body. These lymph tissues include:

  • lymph nodes connected by an extensive network of lymph vessels,
  • the tonsils and adenoids,
  • the spleen which is located in the abdomen just behind and left of the stomach, and
  • the thymus which is a very small organ in the center of the chest behind the upper part of the sternum (breastbone).

Since WBCs are filtered and processed in these tissues, these are the sites where lymphomas develop. As the abnormal WBCs multiply, the lymph tissue swells and can be seen or felt in the neck, above the clavicles (collarbones), under the arms, or in the groin. If the affected lymph tissue is in the abdomen, the swelling may cause symptoms from pressure on other organs or back pain from pressure on nerves.
Within the lymph system are many different cells, each with a specific job to do. For example, some are called lymphocytes and among them are B-cells, T-cells, and NK-cells. Each cell type has something to do with identifying and eliminating foreign particles from the body, such as bacteria, viruses, and damaged cells. Any of the lymphocytes can become cancerous causing one or more subtypes of lymphoma. In fact, cancers of the lymph system represent nearly 40 separate diseases, each with its own name but otherwise similar in their diagnosis, staging, and treatment.

What are the symptoms?
Symptoms of lymphomas vary little from one specific type to another, and in general are rather vague. They may include:

  • Swollen, non-tender lymph nodes
  • Fever
  • Fatigue
  • Nightsweats
  • Weight loss
  • Constant dull backache
  • Itchiness
  • Feeling of fullness in abdomen
  • Pain with alcohol consumption

If a group of these symptoms, especially swollen lymph nodes continues for more than two to three weeks, a physician should be seen. The physician will want to know which symptoms are present and to what degree (when does a fever occur, how often, how long does it last, how high is the fever). Being specific about symptoms helps distinguish more common, less serious disorders from ones for which special tests should be done. Most importantly the explanation for the symptoms should make sense to the doctor and patient. (For example, if a swollen leg is explained as a pulled muscle but the patient is a 75 year old person who does very little activity, the explanation does not fit the person, and further investigation is needed.)

How is the diagnosis made?
A thorough patient history and physical examination are the first and most important steps in making a correct diagnosis. Special attention is paid to feeling for enlarged lymph nodes in the neck, above the collarbones, under the arms and in the groin, and checking the abdomen for an enlarged spleen (upper left side of the abdomen). Blood tests are done to check the number of red blood cells and white blood cells. The blood is tested for the levels of the usual chemicals and enzymes it contains. And a test is done called the "sedimentation rate" which measures how fast blood cells settle to the bottom of a test tube; it is higher in people with serious infections, some immune diseases, and cancers. Standard xrays of the chest and abdomen sometimes show swelling of internal organs or abnormal collections of fluid. Special radiology tests might include CT scans of the chest or abdomen to look for swollen lymph nodes or an enlarged liver or spleen. A bone or gallium scan can show spread to many areas of the body, and an MRI or PET scan can be used to look for disease in certain organs. None of these tests are painful or dangerous and each can provide information about the diagnosis of lymphoma or how far it may have spread.

The final diagnosis of any cancer, including lymphoma, is made from the microscopic examination of cells or tissue (a collection of cells) taken from a part of the body where the disease is thought to be present. The process of getting this tissue is called a biopsy. In the case of lymphoma, a biopsy involves taking tissue from a swollen lymph node or another site that might contain disease. Such a biopsy sometimes can be done with a needle and local anesthetic, but may require an operation under general anesthesia if the tissue is located deep within the body.

In addition to the biopsy, a sample of the bone marrow is taken with a needle from the back of the pelvic bone just above the buttock. Fluid is withdrawn from the space around the spinal cord to look for cancer cells or abnormal amounts of sugar and proteins compared to the levels in the blood. Both of these tests are done using local anesthetic in the skin. All samples of cells or tissues are examined by a pathologist who studies them under a microscope.

Many special tests of the tissues and cells may be needed to make an accurate diagnosis so the biopsy reports can take several days to complete. Sometimes other pathologists may be consulted or more tissue may be needed to make the diagnosis. This can be a difficult time of waiting.

Types of Lymphoma
Why are there so many different names for lymphoma?
Because the immune system has so many different cells and each one goes through several changes in its development, there are many chances that a cell can become abnormal. Doctors who treat lymphoma try to classify all these variations into separate diseases in the hope of finding the best way to care for each one. So even though the number of names for lymphoma seems overwhelming, it just reflects an effort to treat patients as well as possible with the available therapies.
Lymphomas are divided into two major groups, the Hodgkin's lymphomas and the non-Hodgkin's lymphomas. Both are diseases of the lymph or immune system but differ in the age groups affected, the parts of the lymph system involved, the subtypes of cancerous cells, the specific treatments used to combat the disease, and the expected cure and survival rates. Having separated the disease into these two main types, each group is then subdivided into many more specific categories based on the appearance of the cancer cells, the genetic features, chemistry, and clinical behavior of the disease.

Categories of Disease

Non-Hodgkin's Lymphoma
Indolent
B-cell
Follicular
Aggressive
Large cell
T-cell

What is the difference between Indolent and Aggressive non-Hodgkin's lymphoma?


Indolent lymphoma:
Indolent means slow to develop, which is just the case with some NHLs. These types of lymphoma can be difficult to treat because cancer treatments are based on killing cells that are growing rapidly. Often the patient is not made very sick from an indolent NHL and the doctor may take a "wait and watch" approach with the disease. This means checking frequently on a patient's physical exam, blood tests, scans, and sometimes bone marrow to remain alert to the need for treatment if the disease becomes more aggressive. A low-grade (indolent and low-grade are similar names) lymphoma can transform into a different form of lymphoma altogether, always of a more aggressive type. Some indolent lymphomas are treated with radiation alone or with a low-intensity chemotherapy; then these are "watched" in the same way that untreated diseases are.

Aggressive lymphoma:
Lymphoma is usually an aggressive disease, and responds well to chemotherapy and radiation therapy. There are even more subtypes of high-grade lymphomas than of the low-grade diseases. As with the indolent categories, it is an issue of choosing the best treatments for each group of patients that creates all the divisions. Aggressive lymphomas tend to spread to places in the body other than the lymph nodes. The widespread behavior of these diseases, especially the Large Cell lymphomas, makes the outcome of treatment more difficult to predict.

What does the "stage" of disease mean?
Staging of a cancer means determining how far the disease has spread and its level of aggressiveness. The stage helps in the choice of treatment and in anticipating a prognosis.

  • Stage I - disease found in only one site
  • Stage II - more than one site but only on one side of the diaphragm (breathing muscle separating the chest from the abdominal cavity)
  • Stage III - disease in lymph nodes above and below diaphragm
  • Stage IV - disease spread to other organs (lungs, liver, bone marrow, intestinal tract, bone)

B symptoms: if the following symptoms are present with a diagnosis of lymphoma, the patient's stage of disease includes the letter "B."

  • Fever greater than 100.5 F
  • Soaking nightsweats
  • Loss of more than 10% of body weight
  • Tumor larger than 5 inches
  • Disease in more than four sites.

The letter "A" after the stage means these symptoms are Absent. B symptoms represent a more advanced stage of disease in which the prognosis is usually worse.

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