Non-Hodgkin's Lymphomas: The Facts

What is it?

Non-Hodgkin's Lymphomas (NHLs) are a group of Cancers that begin in the lymphatic system. The lymphatic system is a connected network of lymph vessels (similar to blood vessels), lymph nodes, and other structures including the spleen, thymus, and bone marrow. Lymph nodes are small, bean shaped structures that form and store white blood cells to fight infection. Lymph vessels connect lymph nodes throughout the body. The lymphatic system is involved in the body's ability to fight infection and in the formation of blood cells. The various components of this system are spread all over much of the body. For this reason, many other types of cancer (besides Hodgkin's Disease) can eventually spread to the lymphatic system. NHLs can begin in many areas of the body because the lymphatic system is so widespread. As the disease progresses, the cancer can spread throughout the lymphatic system and reach many areas of the body. If the cancer enters the blood stream it can spread to other organs such as the lungs or liver. Non-Hodgkin's Lymphomas are just one category of lymphomas. A lymphoma is any type of cancer that affects white blood cells and the lymphatic system. White blood cells are the cells of the body responsible for fighting infection and disease. There is another type of lymphoma called Hodgkin's Disease. This is a separate disease that will not be discussed here. The main difference between the two is that a special type of immune system cells, called Reed-Sternberg cells, are found in Hodgkin's Disease, but not in NHLs. All lymphomas that are not Hodgkin's disease are grouped together as Non-Hodgkin's Lymphomas. The classification of different stages of NHLs can be very confusing. There are many different forms of the disease and unfortunately, more than one classification system is used to describe them. The majority of doctors use one of the two classification systems described below.

"WORKING FORMULATION"

This system uses descriptive terms based on the appearance of the cancer cells when they are examined microscopically. For example size and shape of the cells are described as follows:

This system is simplified more by grouping together symptoms based on the prognosis. These different categories are generally described with a combination of common and technical language as follows:

• LOW GRADE: Slow growing cancer; symptoms are often not present. This stage can go on for years untreated before problems arise. Characterized by small lymphocytic and follicular, predominantly small cleaved cell or follicular, mixed (small cleaved and large cell).
  
  
• INTERMEDIATE GRADE: Cancer that grows faster than Low Grade; if left untreated, this condition can be fatal in a matter of months. Characterized by follicular, large cell; diffuse, small cleaved cell; diffuse, mixed (small and large cell); or diffuse large cell (cleaved or noncleaved).
  
  
• HIGH GRADE: Very fast growing cancer; if left untreated, this condition can be fatal in a matter of weeks. Characterized by diffuse, large cell iImmunoblastic, lymphoblastic, and small noncleaved cells.
  

"REAL SYSTEM" (Revised European and American Lymphoma Classification System)

This newer system was developed so that doctors all over the world will, hopefully, someday use the same classification scheme. This system classifies stages based on the outlook after treatment, as well as the appearance, chemical makeup, and genetic identity of the cancer cells.

The four stages commonly used in the REAL System are as follows:

• EXCELLENT PROGNOSIS: There is a 5-year survival rate of over 70% for people with this stage. If the cancer is eliminated, then most people survive much longer than this and go on to live cancer-free lives. This category includes anaplastic large T-/Null cell lymphoma; marginal zone B-cell MALT lymphoma, and follicular lymphoma.
  
  
• GOOD PROGNOSIS: There is a 5-year survival rate of about 50% to 70%. This category includes marginal zone B-cell nodal lymphoma; lymphoplasmacytoid lymphoma; Waldenstrom's Macroglobulinemia; and small lymphocytic lymphoma (Chronic Lymphocytic Leukemia).
  
  
• FAIR PROGNOSIS: There is a 5-year survival rate of about 30% to 50%.This category includes primary mediastinal large B-cell lymphoma; diffuse large B-cell lymphoma; Burkitt's lymphoma; and high-grade B-cell Burkitt-like lymphoma.
  
  
• POOR PROGNOSIS: There is a 5-year survival rate below 30%. This category includes peripheral T-cell lymphoma; lymphoblastic lymphoma; and mantle cell lymphoma.
  

Who gets them?

Non-Hodgkin's Lymphoma is the 5th most commonly diagnosed Cancer in the United States. It is estimated that about 56,000 Americans are diagnosed with Non-Hodgkin's Lymphoma yearly. Men are diagnosed about 20% more often than women. Children can sometimes develop NHLs, but the vast majority of cases involve adults. The average age of diagnosis is about 40 years old. Race seems to be a factor in determining who is at risk for NHLs. Although anyone can develop the disease, Caucasians seem to be at a higher risk than African-Americans or Asians. About 26,000 people a year die from NHL. Each year about 460 Rhode Island residents are diagnosed with Non-Hodgkin's Lymphomas. About one quarter of these people will die of the disease.

Over the last few decades, there has been a significant increase in the number of cases of Non-Hodgkin's Lymphomas. The actual cause of this has not been determined. Regardless, NHLs are an increasingly common problem and currently an area of intense research.

A number of risk factors have been identified that increase one's chances of developing a NHL. However, most people that develop NHLs do not have any of these risk factors and those that do have them will not necessarily develop a NHL. Instead, people with one or more of the following risk factors should be especially aware of possible symptoms that develop.

• Age: The risk of NHLs increases as age increases.
  
  
• Sex: NHLs are more common in men than in women.
  
  
• Viruses: Epstein Barr and HTLV-1 virus infections have been associated with NHLs.
  
  
• Weakened Immune System: People with HIV or other conditions that weaken the Immune system (such as immunosuppressant therapy after organ transplantation) have an increased chance of developing all types of lymphomas.
  
  
• Chemical Exposure: Certain chemicals, pesticides, solvents, and fertilizers have been associated with increased risks of cancer.
  

How are they detected?

A number of different tests are performed when a physician suspects that someone may have a Non-Hodgkin's Lymphoma. These tests are used to determine if the person has Cancer, if it is a lymphoma, and the type and stage of the disease. Some of the tests commonly used are listed below

Diagnostic Tests For General Cancer Diagnosis

• Physical Exam: A general physical exam is important, with blood work to rule out other causes of symptoms. Different clusters of lymph nodes are felt and examined for lumps or swelling.
  
  
• Chest X-rays: A type of high-energy radiation used to create images of areas inside the body such as the lungs and bones.
  
  
• CT Scan: A special type of X-ray machine is used to create detailed computer images of areas inside the body.
  
  
• MRI: A powerful type of magnet is used to make computer-generated images of areas inside the body.
  
  
• Bone Scan and Gallium Scan: A radioactive chemical that is attracted to cancer is injected into the bloodstream. Imaging devices are then used to see if there are any cancerous areas on in the bones or any other area of the body. The Gallium test specifically targets cancer that is in the lymphatic system.
  
  
• Lymphangiogram: A special dye is injected into the lymphatic system and x-rays are taken to get images of the entire system.
  

Diagnostic Tests For NHL Diagnosis

• Fine Needle Aspiration Biopsy: A needle is used to remove some fluid and cells from the tumor or lymph node.
  
  
• Surgical Biopsy: A surgeon cuts through the skin and removes an entire lymph node (excisional biopsy) or a piece of a tumor (incisional biopsy). This is later examined in a lab for cancer cells. Local anesthetic is used if the lymph node is near the surface, but general anesthetic is used if it is deeper in the body.
  
  
• Bone marrow aspiration and biopsy: An aspiration involves the removal of a small amount of bone marrow using a needle. A bone marrow biopsy involves the removal of both marrow and a small amount of bone. Both tests are normally done on the hip bone. These tests are used to diagnose the cancer and to determine what stage it is in. A local anesthetic is normally used.
  
  
• Lumbar puncture: Also known as a "spinal tap," In this test a small amount of cerebrospinal fluid (CSF) is removed from the lower back using a thin needle. The fluid is examined for NHL cells. A local anesthetic is normally used.
  

There are different symptoms that have been associated with NHLs. Unfortunately, these symptoms are also associated with many common infections that aren't NHLs. It is impossible to determine if someone has a NHL based only on symptoms alone. If any of the following symptoms persist for more than a few days, then a doctor should be consulted. Remember: experiencing any of the following symptoms does NOT necessarily mean that you have a NHL. Most likely it is a result of an infection, but this also needs to be reported to a physician.

Symptoms:

• A painless swelling in the lymph nodes of the neck, underarm, or groin
  
• Unexplained recurrent fevers or night sweats
  
• Unexplained weight loss
  
• Persistent itchy skin
  
• Coughing or shortness of breath
  
• Fatigue
  
• Decreased appetite
  
• Alcohol Sensitivity - Pain in involved area after alcohol consumption
  

Are they curable?

If caught early, Non-Hodgkin's Lymphomas can be treated and cured. Low Grade NHLs in particular have a high cure rate because they are slow growing. Intermediate and High Grade NHLs must be treated very aggressively in order to stop the cancer. In recent years, many advances have been made that significantly increase the chances of survival at any stage. On average, 70% of people with Low Grade NHLs are alive and cancer free after 10 years. About 70% of people with Intermediate Grade NHLs are alive and cancer free after 5 years. The survival rates for people with High Grade NHLs vary significantly based on the specifics of the disease. Aggressive, fast growing, end stage NHLs are very difficult to treat.

It's easier to prevent cancer than to treat it....

Non-Hodgkin's Lymphomas have been associated with few preventable risk factors; however, there are certain healthy lifestyle habits that can lower your risk of developing other cancers and improve your general health. These include:

• Not smoking
  
• Regular exercise
  
• Moderate, if any, consumption of alcohol
  
• Low fat, high fiber diet
  

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