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Lymphoma

Lymphoma (also termed lymphatic cancer) is a type of cancer involving cells of the immune system, called lymphocytes. Just as cancer represents many different diseases, lymphoma represents many different cancers of lymphocytes -- about 35-60 different subtypes, in fact, depending upon which group of experts is categorizing the subtypes.

Lymphoma is a group of cancers that affect the cells that play a role in the immune system and primarily represents cells involved in the lymphatic system of the body.

The lymphatic system is part of the immune system. It consists of a network of vessels that carry a fluid called lymph, similar to the way that the network of blood vessels carry blood throughout the body. Lymph contains white blood cells called lymphocytes that are also present in blood and tissues. Lymphocytes attack a variety of infectious agents as well as many cells in the precancerous stages of development.

Lymph nodes are small collections of lymph tissue that occur throughout the body. The lymphatic system involves lymphatic channels that connect thousands of lymph nodes scattered throughout the body. Lymph flows through the lymph nodes, as well as through other lymphatic tissues including the spleen, the tonsils, the bone marrow, and the thymus gland.

These lymph nodes filter the lymph, which may carry bacteria, viruses, or other microbes. At infection sites, large numbers of these microbial organisms collect in the regional lymph nodes and produce the local swelling and tenderness typical of a localized infection. These enlarged and occasionally confluent collections of lymph nodes (so-called lymphadenopathy) are often referred to as "swollen glands." In some areas of the body (such as the anterior part of the neck), they are often visible when swollen.

Lymphocytes recognize infectious organisms and abnormal cells and destroy them. There are two major subtypes of lymphocytes: B lymphocytes and T lymphocytes, also referred to as B cells and T cells.

B lymphocytes produce antibodies (proteins that circulate through the blood and lymph and attach to infectious organisms and abnormal cells). Antibodies essentially alert other cells of the immune system to recognize and destroy these intruders (also known as pathogens); the process is known as humoral immunity.

T cells, when activated, can kill pathogens directly. T cells also play a part in the mechanisms of immune system control to prevent the system from inappropriate overactivity or underactivity.

After fighting off an invader, some of the B and T lymphocytes "remember" the invader and are prepared to fight it off if it returns.

Cancer occurs when normal cells undergo a transformation whereby they grow and multiply uncontrollably. Lymphoma is a malignant transformation of either B or T cells or their subtypes.

As the abnormal cells multiply, they may collect in one or more lymph nodes or in other lymph tissues such as the spleen.

As the cells continue to multiply, they form a mass often referred to as a tumor.

Tumors often overwhelm surrounding tissues by invading their space, thereby depriving them of the necessary oxygen and nutrients needed to survive and function normally.

In lymphoma, abnormal lymphocytes travel from one lymph node to the next, and sometimes to remote organs, via the lymphatic system.

While lymphomas are often confined to lymph nodes and other lymphatic tissue, they can spread to other types of tissue almost anywhere in the body. Lymphoma development outside of lymphatic tissue is called extranodal disease.

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What Are the Types of Lymphoma?

Lymphomas fall into one of two major categories: Hodgkin's lymphoma (HL, previously called Hodgkin's disease) and all other lymphomas (non-Hodgkin's lymphomas or NHLs).

These two types occur in the same places, may be associated with the same symptoms, and often have similar appearance on physical examination (for example, swollen lymph nodes). However, they are readily distinguishable via microscopic examination of a tissue biopsy sample because of their distinct appearance under the microscope and their cell surface markers.

Hodgkin's disease develops from a specific abnormal B lymphocyte lineage. NHL may derive from either abnormal B or T cells and are distinguished by unique genetic markers.

There are five subtypes of Hodgkin's disease and about 30 subtypes of non-Hodgkin's lymphoma (not all experts agree on the numbers and names of these NHL subtypes).

Because there are so many different subtypes of lymphoma, the classification of lymphomas is complicated (it includes both the microscopic appearance as well as genetic and molecular markers).

Many of the NHL subtypes look similar, but they are functionally quite different and respond to different therapies with different probabilities of cure. For example, the subtype plasmablastic lymphoma is an aggressive cancer that arises in the oral cavity of HIV-infected patients, the follicular subtype is composed of abnormal B lymphocytes, while anaplastic subtype is comprised of abnormal T cells and cutaneous lymphomas localize abnormal T cells in the skin. As previously mentioned, there are over 30 subtypes of NHL with unusual names such as Mantle cell lymphoma, mucosa associated lymphoid tissue (MALT) lymphoma, hepatosplenic lymphoma and hereditary lymphomas. However, the World Health Organization (WHO) suggests there at least 61 types of NHL; subtyping is still a work in progress. However, no matter how many subtypes experts suggest exist, there are too many to discuss in detail in this article. HL subtypes are microscopically distinct, and typing is based upon the microscopic differences as well as extent of disease.

Lymphoma is the most common type of blood cancer in the United States. It is the seventh most common cancer in adults and the third most common in children. Non-Hodgkin's lymphoma is far more common than Hodgkin's lymphoma.

In the United States, about 72,240 new cases of NHL and 8,260 new cases of HL were expected to be diagnosed in 2017.

About 20,140 deaths due to NHL were expected in 2017, as well as 1,070 deaths due to HL, with the survival rate of all but the most advanced cases of HL greater than that of other lymphomas.

Lymphoma can occur at any age, including childhood. Hodgkin's disease is most common in two age groups: young adults 16-34 years of age and in older people 55 years of age and older. Non-Hodgkin's lymphoma is more likely to occur in older people.

Lymphoma Symptom

Enlarged Spleen (Splenomegaly)

The causes of splenomegaly vary widely and range from malignancy (cancers), infections, congestion (increased blood flow), infiltration of the spleen from other diseases, inflammatory conditions, and blood cell diseases.

Some of the most common causes of an enlarged spleen include the following:

liver disease (cirrhosis due to chronic hepatitis B, chronic hepatitis C, fatty liver, long standing alcohol abuse);

blood cancers (lymphoma, leukemia, myelofibrosis);

infections (mononucleosis, bacterial endocarditis, malaria, AIDS, mycobacterium, leishmania);

abnormal blood flow and congestion (splenic vein thrombosis, portal vein obstruction, congestive heart failure);

Gaucher disease (a lipid storage disease);

blood cell disorders (sickle cell anemia, thalassemia, spherocytosis);

inflammatory disease (lupus, rheumatoid arthritis);

idiopathic thrombocytopenic purpura (ITP); and

polycythemia vera.

Read more about splenomegaly »

What Are Lymphoma Causes and Risk Factors?

The exact causes of lymphoma are not known. Several factors have been linked to an increased risk of developing lymphoma, but it is unclear what role they play in the actual development of lymphoma. These risk factors include the following:

Age: Generally, the risk of NHL increases with advancing age. HL in the elderly is associated with a poorer prognosis than that observed in younger patients. In the 20-24-year age group, the incidence of lymphoma is 2.4 cases per 100,000 while it increases to 46 cases per 100,000 among individuals 60-64 years of age.

Infections

Infection with HIV

Infection with Epstein-Barr virus (EBV), one of the causative factors in mononucleosis, is associated with Burkitt lymphoma, a NHL that most often occurs in children and young people (ages 12 to 30).

Infection with Helicobacter pylori, a bacterium that lives in the digestive tract

Infection with hepatitis B or hepatitis C virus

Medical conditions that compromise the immune system

HIV

Autoimmune disease

Use of immune suppressive therapy (often used following organ transplant)

Inherited immunodeficiency diseases (severe combined immunodeficiency, ataxia telangiectasia, among a host of others)

Exposure to toxic chemicals

Farm work or an occupation with exposure to certain toxic chemicals such as pesticides, herbicides, or benzene and/or other solvents

Hair dye use has been linked to higher rates of lymphoma, especially in patients who started to use the dyes before 1980.

Genetics: Family history of lymphoma

The presence of these risk factors does not mean a person will actually develop lymphoma. In fact, most people with one or several of these risk factors do not develop lymphoma.

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What Are Lymphoma Symptoms and Signs?

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Often, the first sign of lymphoma is a painless swelling of lymph nodes in the neck, under an arm, or in the groin.

Lymph nodes and/or tissues elsewhere in the body may also swell. The spleen, for example, may become enlarged in lymphoma.

The enlarged lymph node sometimes causes other symptoms by pressing against a vein or lymphatic vessel (swelling of an arm or leg), a nerve (pain, numbness, or tingling), or the stomach (early feeling of fullness).

Enlargement of the spleen (splenomegaly) may cause abdominal pain or discomfort.

Many people have no other symptoms.

Symptoms of lymphoma may vary from patient to patient and may include one or more the following:

Fevers

Chills

Unexplained weight loss

Night sweats

Lack of energy

Itching (up to 25% of patients develop this itch [pruritus], most commonly in the lower extremity but it can occur anywhere, be local, or spreading over the whole body)

Loss of appetite

Shortness of breath

Lymphedema

Back or bone pain

Neuropathy

Blood in the stool or vomit

Blockage of urine flow

Headaches

Seizures

These symptoms are nonspecific, and not every patient will have all of these potential symptoms. This means that a patient's symptoms could be caused by any number of conditions unrelated to cancer. For instance, they could be signs of the flu or other viral infection, but in those cases, they would not last very long. In lymphoma, the symptoms persist over time and cannot be explained by an infection or another disease.

When Should Someone Seek Medical Care for Lymphoma?

Swelling in the neck, armpit, or groin, or unexplained swelling in an arm or leg should prompt one to seek medical attention. Such swellings may have many causes or have nothing to do with lymphoma, but they should be checked.

If any of the following symptoms persist for more than a few days, seek medical attention:

Fevers

Chills

Unexplained weight loss

Night sweats

Lack of energy

Itching

What Tests Do Doctors Use to Diagnose Lymphoma?

If a person has swelling or symptoms described in the Symptoms section, his or her health-care provider will ask many questions about the symptoms (when they began, recent illnesses, past or current medical problems, any medications, workplace, health history, family history, and habits and lifestyle). These questions are followed by a thorough examination.

If, after an initial interview and examination, the health-care provider suspects that a patient may have lymphoma, the patient will undergo a series of tests designed to provide further clarification. At some point in this workup, the patient might be referred to a specialist in blood diseases and cancer (hematologist/oncologist).

Blood tests

Blood is drawn for various tests.

Some of these tests evaluate the function and performance of blood cells and important organs, such as the liver and kidneys.

Certain blood chemicals or enzymes (lactate dehydrogenase [LDH]) may be determined. High levels of LDH in cases in which NHL is suspected may indicate a more aggressive form of the disorder.

Other tests may be done to learn more about lymphoma subtypes.

Biopsy

If there is a swelling (also called lump or mass), a sample of tissue from the swelling will be removed for examination by a pathologist. This is called a biopsy. Any of several methods can be used to obtain a biopsy of a mass.

Masses that can be seen and felt under the skin are relatively easy to biopsy. A hollow needle can be inserted into the mass and a small sample removed with the needle (called a core-needle biopsy). This is usually done in the health-care provider's office with a local anesthetic.

Core-needle biopsy does not always obtain a good-quality sample. For that reason, many health-care providers prefer a surgical biopsy. This involves removal of the entire swollen lymph node through a small incision in the skin. This procedure often is done with a local anesthetic, but it sometimes requires a general anesthetic.

If the mass is not immediately under the skin but is instead deep inside the body, access is somewhat more complicated. The tissue sample is usually obtained via laparoscopy. This means making a tiny incision in the skin and inserting a thin tube with a light and a camera on the end (a laparoscope). The camera sends pictures of the inside of the body to a video monitor, and the surgeon can see the mass. A small cutting tool on the end of the laparoscope can remove all or part of the mass. This tissue is withdrawn from the body with the laparoscope.

A pathologist (a physician who specializes in diagnosing diseases by looking at cells and tissues) examines the tissue sample with a microscope. The pathologist's report will specify whether the tissue is lymphoma and the type and subset of lymphoma.

Imaging studies

If there is no palpable mass in the presence of persistent symptoms, imaging studies will likely be carried out in order to determine whether a mass is present and, if so, how then to direct a biopsy.

X-rays: In certain parts of the body, such as the chest, a simple X-ray can sometimes detect lymphoma.

CT scan: This test provides a three-dimensional view and much greater detail and may detect enlarged lymph nodes and other masses anywhere in the body.

MRI scan: Similar to the CT scan, MRI gives three-dimensional images with excellent detail. MRI provides better definition than CT scan in certain parts of the body, especially the brain and the spinal cord.

Positron-emission tomographic (PET) scan: PET scan is a newer alternative to lymphangiogram and gallium scan for detecting areas in the body that are affected by lymphoma. A tiny amount of a radioactive substance is injected into the body and then traced on the PET scan. Sites of radioactivity on the scan indicate areas of increased metabolic activity, which implies the presence of a tumor.

Bone marrow examination

Most of the time, an examination of the bone marrow is necessary to see if the marrow is affected by the lymphoma. This is done by collecting a biopsy of the bone marrow.

Samples are taken, usually from the pelvis bone.

A pathologist examines the bone marrow under a microscope.

Bone marrow containing certain types of abnormal B or T lymphocytes confirms lymphoma.

Bone marrow biopsy can be an uncomfortable procedure, but it can usually be performed in a medical office. Most people receive pain medication prior to the procedure to make them more comfortable.

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How Is Lymphoma Staging Determined?

Staging is the classification of a cancer type by its size and whether and how much it has spread around the body. Determining a cancer's stage is very important because it tells the oncologist which treatment is most likely to work and what are the chances of remission or a cure (prognosis).

Staging of lymphomas is based on the results of imaging studies and related tests that reveal the extent of the cancer involvement.

HL is often described as being "bulky" or "nonbulky." Nonbulky means the tumor is small; bulky means the tumor is large. Nonbulky disease has a better prognosis than bulky disease.

NHL is a complicated set of diseases with a complex classification system. In fact, the classification system is continuously evolving as we learn more about these cancers. The newest classification system takes into account not only the microscopic appearance of the lymphoma but also its location in the body and genetic and molecular features.

Grade is also an important component of the NHL classification.

Low grade: These are often called "indolent" lymphomas because they grow slowly. Low-grade lymphomas are often widespread when discovered, but because they grow slowly, they usually do not require immediate treatment unless organ function is compromised. They are rarely cured and can transform over time to a combination of indolent and aggressive types.

Intermediate grade: These are rapidly growing (aggressive) lymphomas that usually require immediate treatment, but they are often curable.

High grade: These are very rapidly growing and aggressive lymphomas that require immediate, intensive treatment and are much less often curable.

The "staging," or evaluation of extent of disease, for both HL and NHL, are similar.

Stage I (early disease): Lymphoma is located in a single lymph node region or in one lymphatic area.

Stage IE: Cancer is found in one area or organ outside the lymph node.

Stage II (early disease): Lymphoma is located in two or more lymph node regions all located on the same side of the diaphragm.

Stage IIE: As II, but cancer is also found outside the lymph nodes in one organ or area on the same side of the diaphragm as the involved lymph nodes. (The diaphragm is a flat muscle that separates the chest from the abdomen.)

Stage III (advanced disease): Lymphoma affects two or more lymph node regions, or one lymph node region and one organ, on opposite sides of the diaphragm.

Stage IV (widespread or disseminated disease): Lymphoma is outside the lymph nodes and spleen and has spread to another area or organ such as the bone marrow, bone, or central nervous system.

If the cancer is also found in the spleen, an "S" is added to the classification.

Prognostic factors

Several risk factors have been extensively evaluated and shown to play a role in treatment outcome. For HL, the International Prognostic Index includes the following seven risk factors:

Male sex

Age 45 years or older

Stage IV disease

Albumin (blood test) less than 4.0 g/dL

Hemoglobin (red blood cell level) less than 10.5 g/dL

Elevated white blood cell (WBC) count of 15,000/mL

Low lymphocyte count less than 600/mL or less than 8% of total WBC

The absence of any of the above risk factors is associated with an 84% rate of control of Hodgkin's disease, whereas the presence of a risk factor is associated with a 77% rate of disease control. The presence of five or more risk factors was associated with a disease control rate of only 42%.

The outcomes for these patients were also determined by the treatment they received, which occurred primarily in the 1980s. Newer treatments for Hodgkin's lymphoma may improve these predicted outcomes. Furthermore, new treatments are being developed for patients with greater risk factors.

The International Prognostic Index for NHL includes five risk factors:

Age older than 60 years

Stage III or IV disease

High LDH

More than one extranodal site

Poor performance status (as a measure of general health): From these factors, the following risk groups were identified:

Low risk: no or one risk factor, has a five-year overall survival of approximately 73%

Low-intermediate risk: two risk factors, has a five-year overall survival of approximately 50%

High-intermediate risk: three risk factors, has a five-year overall survival of approximately 43%

High risk: four or more risk factors, has a five-year overall survival of approximately 26%

The prognostic models were developed to evaluate groups of patients and are useful in developing therapeutic strategies. It is important to remember that any individual patient might have significantly different results than the above data, which represent statistical results for a patient group. There are specific IPIs for certain types of lymphoma, such as follicular or diffuse large B-cell.

What Types of Doctors Treat Lymphoma?

Although the patient's primary-care doctor or pediatrician can help manage the patient's care, other specialists are usually involved as consultants. Oncologists, hematologists, pathologists, and radiation oncologists are usually involved in making treatment plans and caring for the patient. Occasionally, other specialists may need to be involved depending upon what organs may be at risk in the individual's disease process.

What Is the Treatment for Lymphoma?

General health-care providers rarely undertake the sole care of a cancer patient. The vast majority of cancer patients receive ongoing care from oncologists but may in fact be referred to more than one oncologist should there be any question about the disease. Patients are always encouraged to gain second opinions if the situation so warrants this approach.

One may choose to speak with more than one oncologist to find the one with whom he or she feels most comfortable.

In addition to one's primary-care physician, family members or friends may offer information. Also, many communities, medical societies, and cancer centers offer telephone or Internet referral services.

Once one settles in with an oncologist, there is ample time to ask questions and discuss treatment regimens.

The doctor will present each type of treatment, discuss the pros and cons, and make recommendations based on published treatment guidelines and his or her own experience.

Treatment for lymphoma depends on the type and stage. Factors such as age, overall health, and whether one has already been treated for lymphoma before are included in the treatment decision-making process.

The decision of which treatment to pursue is made with the doctor (with input from other members of the care team) and family members, but the decision is ultimately the patient's.

Be certain to understand exactly what will be done and why and what can be expected from these choices.

As in many cancers, lymphoma is most likely to be cured if it is diagnosed early and treated promptly.

The most widely used therapies are combinations of chemotherapy and radiation therapy.

Biological therapy, which targets key features of the lymphoma cells, is used in many cases nowadays.

The goal of medical therapy in lymphoma is complete remission. This means that all signs of the disease have disappeared after treatment. Remission is not the same as cure. In remission, one may still have lymphoma cells in the body, but they are undetectable and cause no symptoms.

When in remission, the lymphoma may come back. This is called recurrence.

The duration of remission depends on the type, stage, and grade of the lymphoma. A remission may last a few months, a few years, or may continue throughout one's life.

Remission that lasts a long time is called durable remission, and this is the goal of therapy.

The duration of remission is a good indicator of the aggressiveness of the lymphoma and of the prognosis. A longer remission generally indicates a better prognosis.

Remission can also be partial. This means that the tumor shrinks after treatment to less than half its size before treatment.

The following terms are used to describe the lymphoma's response to treatment:

Improvement: The lymphoma shrinks but is still greater than half its original size.

Stable disease: The lymphoma stays the same.

Progression: The lymphoma worsens during treatment.

Refractory disease: The lymphoma is resistant to treatment.

The following terms to refer to therapy:

Induction therapy is designed to induce a remission.

If this treatment does not induce a complete remission, new or different therapy will be initiated. This is usually referred to as salvage therapy.

Once in remission, one may be given yet another treatment to prevent recurrence. This is called maintenance therapy.

Medical Treatment: Radiation and Chemotherapy

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Standard first-line therapy (primary therapy) for lymphoma includes radiation therapy for most early-stage lymphomas, or a combination of chemotherapy and radiation. For later-stage lymphomas, chemotherapy is primarily used, with radiation therapy added for control of bulky disease. Biological therapy, or immunotherapy, is routinely used alongside chemotherapy.

Radiation therapy

Radiation therapy uses high-energy rays to kill cancer cells. It is considered a local therapy, meaning that it should be used to target areas of the body involved by tumor masses. A radiation oncologist will plan and supervise therapy.

The radiation is targeted at the affected lymph node region or organ. Occasionally, nearby areas are also irradiated to kill any cells that might have spread there undetected.

Depending on how and where the radiation is administered, it may cause certain side effects such as fatigue, loss of appetite, nausea, diarrhea, and skin problems. Radiation of lymph node areas may result in suppression of the immune system to varying degrees. Irradiation of the underlying bone and the marrow within the bone may result in suppression of the blood counts.

The radiation is usually administered in short bursts on five days a week over the course of several weeks. This keeps the dose of each treatment low and helps prevent or lessen side effects.

Chemotherapy

Chemotherapy is the use of powerful drugs to kill cancer cells. Chemotherapy is a systemic therapy, meaning that it circulates through the bloodstream and affects all parts of the body.

Unfortunately, chemotherapy also affects healthy cells; this accounts for its well-known side effects.

The side effects of chemotherapy depend partly on the drugs used and the doses.

Some people, because of variability in metabolism of chemotherapy drugs, tolerate chemotherapy better than other people.

The most common side effects of chemotherapy include suppression of blood counts, which could result in increased susceptibility to infection (low white blood cell count), anemia (low red blood cell count that may require a blood transfusion), or blood-clotting problems (low platelet count). Other side effects may include nausea and vomiting, loss of appetite, hair loss, sores in the mouth and digestive tract, fatigue, muscle aches, and changes in fingernails and toenails.

Medications and other treatments are available to help people tolerate these side effects, which can be severe.

It is very important to discuss and review the potential side effects of each chemotherapy drug in the treatment with the oncologist, pharmacist, or oncology nurse. Medications to lessen the side effects should also be reviewed.

Chemotherapy may be given in pill form, but it is typically a liquid infused directly into the bloodstream through a vein (intravenous).

Most people who receive intravenous chemotherapy will have a semi-permanent device placed in a large vein, usually in the chest or arm.

This device allows the medical team quick and easy access to the blood vessels, both for administering medications and for collecting blood samples.

These devices come in several types, usually referred to as a "catheter," "port," or "central line."

Experience has shown that combinations of drugs are more efficient than monotherapy (use of a single medication).

Combinations of different drugs both increase the chance that the drugs will work and lower the dose of each individual drug, reducing the chance of intolerable side effects.

Several different standard combinations are used in lymphoma. Which combination one receives depends on the type of lymphoma and the experiences of the oncologist and the medical center where a person is receiving treatment.

The combinations of drugs are usually given according to a set schedule that must be followed very strictly.

In some situations, chemotherapy can be given in the oncologist's office. In other situations, one must stay in the hospital.

Chemotherapy is given in cycles.

One cycle includes the period of actual treatment, usually several days, followed by a period of rest for several weeks to allow recovery from side effects caused by the chemotherapy, particularly anemia and low white blood cells.

Standard treatment typically includes a set number of cycles, such as four or six.

Spreading the chemotherapy out this way allows a higher cumulative dose to be given, while improving the person's ability to tolerate the side effects.

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Medical Treatment: Biological Therapy

Biological therapies are sometimes referred to as immunotherapy because they take advantage of the body's natural immunity against pathogens. These therapies are attractive because they offer anticancer effects without many of the undesirable side effects of standard therapies. There are many different types of biological therapies. The following are some of the most promising for treating lymphoma:

Monoclonal antibodies: Antibodies are substances produced by our body to fight pathogens. Every cell, organism, or pathogen within our body carries markers on its surface that antibodies may recognize. These surface markers are called antigens. A monoclonal antibody is an antibody that is made in a laboratory to find and attach itself to a specific antigen. Monoclonal antibodies can be used to help one's own immune systems kill tumor cells and other pathogens directly, or they can deliver cancer-killing therapies (such as radiation or chemotherapy) directly to a specific antigen found on cancer cells.

Cytokines: These naturally occurring chemicals are produced by the body to stimulate the cells in the immune system and other organs. They can also be produced artificially and administered in large doses to patients with greater effect. Examples include interferons and interleukins, which stimulate the immune system, and colony-stimulating factors, which stimulate the growth of blood cells.

Vaccines: Unlike the more familiar vaccines for infectious diseases such as polio and flu, cancer vaccines do not prevent the disease. Rather, they are designed to stimulate the immune system to mount a specific response against the cancer. They also create a "memory" of the cancer so that the immune system activates very early in cases of recurrence, thus preventing the development of a new tumor.

Other therapies that are primary or supportive medications are undergoing continuing development and refinement; they include drugs that target cancer cells on a molecular level, various new monoclonal antibodies, and other biologic therapies such as steroids and bone marrow stimulants.

What Other Therapies Treat Lymphoma?

Watchful waiting means choosing to observe and monitor the cancer rather than treat it right away. This is strategy is sometimes used for indolent recurrent tumors. Treatment is given only if the cancer starts to grow more rapidly or to cause symptoms or other problems.

Stem cell transplantation is usually not used as a primary therapy in lymphoma.

Stem cell transplantation is usually reserved for lymphoma that has been previously treated into remission but has recurred.

Stem cell therapy as primary therapy has been used for aggressive T-cell NHL in first remission, usually as part of a clinical trial. Stem cell therapy is also considered when standard primary treatment is unable to control the lymphoma and achieve remission.

This procedure, which requires a lengthy hospital stay, involves very high doses of chemotherapy to kill the aggressive cancer cells.

The chemotherapy doses are so high that the chemotherapy also stops the patient's bone marrow from producing healthy new blood cells.

The patient then receives a transfusion of healthy bone marrow or blood stem cells, either from previously collected stem cells from the patient himself or herself (called autologous transplantation or autotransplantation) or from a donor (called allogeneic transplantation), to "kick start" the bone marrow into producing healthy blood cells.

This is a very intensive therapy with a lengthy recovery period.

Clinical trials

An oncologist may belong to a network of investigators who offer novel treatments for various types of cancers. These novel therapies are newer agents that have been recently developed, and extensive data on treatment results are not yet known. Such new agents can be offered in the context of a clinical trial. Usually, a consent form that explains the drug, its known side effects, its potential side effects, and alternatives to the drug treatment are presented to the patient. If the treatment seems promising for the particular subtype of lymphoma, and the patient is fully educated about the potential benefits and risks associated with such treatment and is interested in receiving such treatment, then the consent form is signed by the patient and the managing physician, and possibly other involved parties. The patient is then enrolled in a treatment protocol that specifies exactly how a patient is to be treated with the new therapy.

Alternatively, an oncologist may refer a patient to another institution to receive investigational treatment or intensive treatment, such as a stem cell transplant.

Complementary/alternative therapies

Several alternative therapies have undergone preliminary testing in lymphoma. None has been found to work better than or as well as standard medical therapies. A few therapies, still felt to be experimental, have been found to be potentially helpful as complements to medical therapy, however.

Acupuncture has been helpful in relieving musculoskeletal symptoms, as well as in controlling nausea and vomiting associated with chemotherapy.

The supplements coenzyme Q10 and polysaccharide K (PSK) are undergoing further evaluation to determine their effects on treatment outcome. Both drugs have immune enhancing properties. PSK has been used more extensively in Japan as part of an anticancer therapy.

Neither of these therapies has undergone extensive blinded studies and cannot be considered a part of any planned therapeutic endeavor.

Do not use these therapies unless you first discuss them with your doctor.

What Medications Treat Lymphoma?

Numerous chemotherapy and biological drug combinations may be prescribed by an oncologist. Which type and combination of therapy depends on many factors, including the type and stage of lymphoma, the age of the patient, the ability to tolerate chemotherapy side effects, and if any previous treatment for the lymphoma has occurred. Oncologists often work together regionally to decide which combination of chemotherapy and biological drugs are currently working best for their patients. Because of this regional collaboration, the drug combinations often vary and are able to change rapidly when improved results occur.

What Follow-up May Be Needed After Lymphoma Treatment?

After completion of primary therapy for lymphoma, all appropriate tests will be repeated to see how well the therapy worked.

The results of these tests will tell the oncologist whether the patient is in remission.

If the patient is in remission, the oncologist will recommend a schedule of regular testing and follow-up visits to monitor the remission and to catch any recurrence early.

Keeping up with these visits and tests is very important in order to avoid advanced or widespread disease.

If lymphoma recurs after treatment, the oncologist will likely recommend further treatment.

What Is the Prognosis of Lymphoma?

The outlook for HL is very good. It is one of the most curable cancers. The five-year survival rate after treatment is greater than 80% for adults and greater than 90% for children.

As a result of refinements in and more aggressive approaches to therapy, the outlook for NHL has improved significantly in the last few decades. The five-year survival rate after treatment is 69% for adults and up to 90% for children; the 10-year relative survival rate is 59%. The addition of immunotherapy to standard treatment for NHLs may further improve survival rates so that life expectancy may move toward normal.

Many people live with lymphoma in remission for many years after treatment.

Is It Possible to Prevent Lymphoma?

There is no known way to prevent lymphoma. A standard recommendation is to avoid the known risk factors for the disease. However, some risk factors for lymphoma are unknown, and therefore impossible to avoid. Infection with viruses such as HIV, EBV, and hepatitis are risk factors that can be avoided with frequent hand washing, practicing safe sex, and by not sharing needles, razors, toothbrushes, and similar personal items that might be contaminated with infected blood or secretions.

Support Groups and Counseling for Lymphoma

Living with lymphoma presents many new challenges for an individual and his or her family and friends.

There may be many worries about how the lymphoma will affect one's ability to "live a normal life," that is, to care for family and home, to hold a job, and to continue the friendships and activities one enjoys.

Many people feel anxious and depressed. Some people feel angry and resentful; others feel helpless and defeated.

For most people with lymphoma, talking about their feelings and concerns can be helpful.

Friends and family members can be very supportive. They may be hesitant to offer support until they see how the affected person is coping. If the affected person wishes to talk about his or her concerns, it is important to let them know to do so.

Some people don't want to "burden" their loved ones, or they prefer talking about their concerns with a more neutral professional. A social worker, counselor, or member of the clergy can be helpful if one wishes to discuss their feelings and concerns about having lymphoma. The treating hematologist or oncologist should be able to recommend someone.

Many people with lymphoma are helped profoundly by talking to other people who have lymphoma. Sharing such concerns with others who have been through the same thing can be remarkably reassuring. Support groups of people with lymphoma may be available through the medical center where one is receiving treatment. The American Cancer Society also has information about support groups all over the United States.

Reviewed on 9/11/2017

REFERENCES:

American Cancer Society. "Non-Hodgkin Lymphoma." Jan. 22, 2016. <http://www.cancer.org/cancer/non-hodgkinlymphoma/detailedguide/non-hodgkin-lymphoma-types-of-non-hodgkin-lymphoma>.

American Cancer Society. "What Are the Key Statistics About Non-Hodgkin Lymphoma?" Jan. 6, 2017. <https://www.cancer.org/cancer/non-hodgkin-lymphoma/about/key-statistics.html>.

"Clinical presentation and diagnosis of non-Hodgkin lymphoma." UptoDate.com

"Epidemiology, pathologic features, and diagnosis of classical Hodgkin lymphoma." UptoDate.com

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Secondary cancer in the lymph nodes

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Sometimes cancer cells can spread from where the cancer started. They may travel through the lymphatic system to lymph nodes in another part of the body.

Symptoms depend on which lymph nodes are affected. The most common symptom is enlarged or swollen lymph nodes. Other symptoms might include breathlessness or backache, for example if there are enlarged lymph nodes deep inside the chest or tummy. Sometimes there are no symptoms and the secondary cancer might be picked up by a routine scan.

You may have a computerised tomography (CT) scan, magnetic resonance imaging (MRI) scan, or positron emission tomography (PET) scan to diagnose secondary cancer in the lymph nodes. Some people will also have a lymph node biopsy.

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Lymph Node Status

What are lymph nodes?

Lymph nodes are small clumps of immune cells that act as filters for the lymphatic system. The lymphatic system runs throughout the body (like the circulatory (blood) system) and carries fluid and cells.

If breast cancer spreads, the lymph nodes in the underarm (the axillary lymph nodes) are the first place it’s likely to go.

Figure 4.4 shows the location of the axillary lymph nodes.

Axillary lymph nodes

What is lymph node status?

Lymph node status is highly related to prognosis.

Lymph node-negative means the axillary lymph nodes do not contain cancer.

Lymph node-positive means the axillary lymph nodes contain cancer.

Prognosis is better when cancer has not spread to the lymph nodes (lymph node-negative) [9].

The more lymph nodes that contain cancer, the poorer prognosis tends to be [9].

 

For a summary of research studies on lymph node status and breast cancer survival, visit the Breast Cancer Research Studies section.

Learn about lymph node status and breast cancer stage.

Lymph node status and tumor size

Lymph node status is related to tumor size.

The larger the tumor, the more likely it is the breast cancer has spread to the lymph nodes (lymph node-positive) [10].

Physical exams and pathology exams

Positive lymph nodes can sometimes be felt during a physical exam, but a pathologist’s exam of the lymph nodes removed during a biopsy is needed to determine lymph node status.

During a physical exam, a health care provider feels under the arm to check if the lymph nodes are enlarged. If the lymph nodes feel enlarged, it’s likely the cancer has spread. However, if the lymph nodes don’t feel enlarged, it doesn’t mean the nodes are negative (cancer-free).

The pathologist checks the nodes under a microscope. Nearly one-third of women with negative lymph nodes based on a physical exam have nodes with cancer found during the pathology exam [10]. And, some women with enlarged nodes during a physical exam have cancer-free nodes [10].

Assessing lymph nodes (sentinel node biopsy and axillary dissection)

Sentinel node biopsy is the most common way to check the axillary lymph nodes for cancer.

In some cases, an axillary dissection (which removes more tissue than a sentinel node biopsy) is advised.

Learn more about sentinel node biopsy, axillary dissection and lymph node assessment.

 

For a summary of research studies on sentinel node biopsy and lymph node status, visit the Breast Cancer Research Studies section.

Topics under study

At this time, sentinel node biopsy is not recommended for people who have locally advanced or inflammatory breast cancer [11-12].

However, the accuracy of sentinel node biopsy in these cases is under study.

Updated 10/28/17

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Lymph nodes

Lymph nodes are small, bean-shaped organs which produce and store blood cells that help fight disease and infection. Also known as lymph glands, lymph nodes remove cell waste and fluids from lymph (lymphatic fluid), and store lymphocytes (white blood cells). Lymph nodes are part of the lymphatic system and are located throughout the body, including the neck, armpits, abdomen and groin.

Causes of swollen lymph nodes

There are many causes for swollen lymph nodes. Most commonly, lymph nodes become swollen due to an infection, such as a viral upper respiratory infection like the common cold. Sometimes, lymph node swelling is caused by an underlying condition. When lymph node swelling persists and is accompanied by other symptoms, such as fever, night sweats or weight loss, without any obvious infection, it may be time to see a doctor for an evaluation.

Cancer in the lymph nodes

Some cancers can cause swelling of the lymph nodes. Cancer may start in the lymph nodes or, more commonly, it spreads there from somewhere else.

Pain or swelling in the area of the lymph nodes is a common symptom of cancer that starts in the lymphatic system, such as non-Hodgkin lymphoma and Hodgkin lymphoma.

Cancer that starts in another part of the body and spreads to the lymph nodes is called a metastasis. Even when cancer spreads to the lymph nodes, it is still named after the area of the body where it started. Learn more about metastatic cancer, including diagnosis and treatment.

View a complete list of cancers we treat

Lymph nodes and staging cancer

Lymph nodes play an important role in cancer staging, which determines the extent of cancer in the body. One of the most commonly used systems for staging cancer is the TNM system, which is based on the extent of the tumor (T), the extent of spread to the lymph nodes (N), and the presence of metastasis (M).

If there’s no cancer found in the lymph nodes near the cancer, the N is assigned a value of 0. If nearby or distant nodes show cancer, the N is assigned a number (such as 1, 2 or 3), depending on how many nodes are affected, how much cancer is in them, how large they are, and where they are.

Treatment for cancer in the lymph nodes

Treatment for cancer in the lymph nodes depends on a variety of factors, including tumor size and location, and whether or not the cancer has metastasized (spread) to other areas of the body.

Surgery may be used to treat some forms of metastatic cancer that have spread to the lymph nodes. Other lymph node cancer treatment options may include chemotherapy, stem cell transplantation, and other cancer therapies.

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Lymph Nodes and Cancer

What is the lymph system?

Our bodies have a network of lymph vessels and lymph nodes. (Lymph is pronounced limf.) This network is a part of the body’s immune system. It collects fluid, waste material, and other things (like viruses and bacteria) that are in the body tissues, outside the bloodstream.

Lymph vessels are a lot like the veins that collect and carry blood through the body. But instead of carrying blood, these vessels carry the clear watery fluid called lymph.

color diagram showing the lymphatic system in the human body (location of lymph nodes, lymphatic vessels)/also shows the location of the thymus, spleen, bone marrow, stomach, colon and small intestine

Lymph fluid flows out from capillary walls to bathe the body’s tissue cells. It carries oxygen and other nutrients to the cells, and carries away waste products like carbon dioxide (CO2) that flow out of the cells. Lymph fluid also contains white blood cells, which help fight infections.

Lymph fluid would build up and cause swelling if it were not drained in some way. That’s the role of the lymph vessels. Lymph vessels draw up the lymph fluid from around the cells to send it towards the chest. There, lymph fluid collects into a large vessel that drains into a blood vessel near the heart.

Lymph nodes and what they do

Lymph vessels route lymph fluid through nodes throughout the body. Lymph nodes are small structures that work as filters for harmful substances. They contain immune cells that can help fight infection by attacking and destroying germs that are carried in through the lymph fluid.

There are hundreds of lymph nodes throughout the body. Each lymph node filters the fluid and substances picked up by the vessels that lead to it. Lymph fluid from the fingers, for instance, works its way toward the chest, joining fluid from the arm. This fluid may filter through lymph nodes at the elbow, or those under the arm. Fluid from the head, scalp, and face flows down through lymph nodes in the neck. Some lymph nodes are deep inside the body, such as between the lungs or around the bowel, to filter fluid in those areas. The lymph fluid slowly flows in from all around the body, making its way back to the chest. At the end of its journey, the filtered fluid, salts, and proteins are dumped back into the bloodstream.

Swollen lymph nodes

When there’s a problem, such as infection, injury, or cancer, the node or the group of lymph nodes in that area may swell or enlarge as they work to filter out the “bad” cells. This may be called lymphadenopathy (LIMF-ad-uh-NOP-uh-thee). Swollen lymph nodes tell you that something is not right, but other symptoms help pinpoint the problem. For instance, ear pain, fever, and enlarged lymph nodes near your ear are clues that you may have an ear infection or cold.

Some areas where lymph nodes commonly swell are in the neck, groin, and underarms. In most cases, only one area of nodes swells at a time. When more than one area of lymph nodes is swollen it’s called generalized lymphadenopathy. Some infections (such as strep throat and chicken pox), certain medicines, immune system diseases, and cancers like lymphoma and leukemia can cause this kind of swelling. The health care provider will look for more information to figure out the cause of the swelling. Lymph node swelling is often caused by something other than cancer.

Cancer in the lymph nodes

Cancer can appear in the lymph nodes in 2 ways: it can either start there or it can spread there from somewhere else.

Cancer that starts in the lymph nodes is called lymphoma. You can read more about lymphoma in Hodgkin Disease and Non-Hodgkin Lymphoma.

More often, cancer starts somewhere else and then spreads to lymph nodes. That is the focus of this section.

How does cancer spread to lymph nodes?

Cancer can spread from where it started (the primary site) to other parts of the body.

When cancer cells break away from a tumor, they can travel to other areas of the body through either the bloodstream or the lymph system. Cancer cells can travel through the bloodstream to reach distant organs. If they travel through the lymph system, the cancer cells may end up in lymph nodes. Either way, most of the escaped cancer cells die or are killed before they can start growing somewhere else. But one or two might settle in a new area, begin to grow, and form new tumors. This spread of cancer to a new part of the body is called metastasis.

In order for cancer cells to spread to new parts of the body, they have to go through several changes. They first have to become able to break away from the original tumor and then attach to the outside wall of a lymph vessel or blood vessel. Then they must move through the vessel wall to flow with the blood or lymph to a new organ or lymph node.

When cancer grows inside lymph nodes, it usually affects the lymph nodes near the tumor itself. These are the nodes that have been doing most of the work to filter out or kill the cancer cells.

How is cancer in lymph nodes found?

Normal lymph nodes are tiny and can be hard to find, but when there’s infection, inflammation, or cancer, the nodes can get larger. Those near the body’s surface often get big enough to feel with your fingers, and some can even be seen. But if there are only a few cancer cells in a lymph node, it may look and feel normal. In that case, the doctor must check for cancer by removing all or part of the lymph node.

When a surgeon operates to remove a primary cancer, one or more of the nearby (regional) lymph nodes may be removed as well. Removal of one lymph node is called a biopsy. When many lymph nodes are removed, it’s called lymph node sampling or lymph node dissection. When cancer has spread to lymph nodes, there’s a higher risk that the cancer might come back after surgery. This information helps the doctor decide whether more treatment, like chemo or radiation, might be needed after surgery.

Doctors may also take samples of one or more nodes using needles. Usually, this is done on lymph nodes that are enlarged. This is called a needle biopsy. The tissue that’s removed is looked at under the microscope by a pathologist (a doctor who diagnoses illness using tissue samples) to find out if there are cancer cells in it

Under the microscope, any cancer cells in the nodes look like the cancer cells from the primary tumor. For instance, when breast cancer spreads to the lymph nodes, the cells in the nodes look like breast cancer cells. The pathologist prepares a report, which details what was found. If a node has cancer in it, the report describes what it looks like and how much was seen.

Doctors may also use scans or other imaging tests to look for enlarged nodes that deep in the body. For more on this, see Imaging (Radiology) Tests. Often, enlarged lymph nodes near a cancer are assumed to contain cancer.

What does it mean if there’s cancer in my lymph node?

It depends. Sometimes there are so few cancer cells in the node that the pathologist must use special tests to find them. In the case of a very few cancer cells in a lymph node, it may not change the treatment plan at all.

If there’s a lot of cancer in a node, the large mass can be seen easily. If the cancer is growing out of the lymph node through the layer of connective tissue on the outside (called the capsule), it’s called extracapsular extension.

More cancer in the nodes may mean that the cancer is fast growing and/or more likely to spread to other places in the body. But if nearby lymph nodes are the only other place cancer is found beyond the main (primary) site, surgery to remove the main tumor and the nearby lymph nodes may be able to get rid of it all.

Cancer that has spread to nodes further away from the primary cancer will more likely need extra treatment with chemo or radiation.

Cancer in nodes affects cancer stage

Treatment of cancer is based on the type of cancer a person has, and the stage of the cancer. Doctors use a system to assign a stage to the cancer. The most common staging system is the TNM system. The T in TNM stands for tumor, the M stands for metastasis, and the N stands for lymph nodes. If there’s no cancer found in the lymph nodes near the cancer, the N is assigned a value of 0. If nearby or distant nodes show cancer, the N is assigned a number such as 1, 2 or sometimes 3, depending on how many nodes are affected, how much cancer is in them, how large they are, and where they are.

A cancer with lower TNM numbers is usually easier to treat and has a better outlook for survival. For instance, a cancer with T1, N0, M0, would be a cancer that was found very early, before it spread. The T1 would mean a small tumor, the N0 means that no nodes are involved, and the M0 means that no metastases were found. For more information on staging, see information about your cancer type, or read Staging.

Effects of removing lymph nodes

Nodes that have been removed during cancer surgery can leave part of the body without a way to drain off the lymph fluid in the affected area. Many of the lymph vessels now run into a dead end where the node used to be, and fluid can back up. This is called lymphedema, and it can become a life-long problem. The more lymph nodes that are removed, the more likely it is to occur. For more on lymphedema, see “To learn more.”

Written by

References

The American Cancer Society medical and editorial content team

Our team is made up of doctors and master's-prepared nurses with deep knowledge of cancer care as well as journalists, editors, and translators with extensive experience in medical writing.

Last Medical Review: April 14, 2015 Last Revised: April 14, 2015

American Cancer Society medical information is copyrighted material. For reprint requests, please see our Content Usage Policy.

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