What Is Lymphoma?
Lymphoma is a cancer of the lymphatic system. The lymphatic system carries lymph
fluid and white blood cells throughout body. The purpose of the lymphatic system is to
fight infections.1,2
Like all cancers, lymphoma happens when the body’s cells grow out of control, often
causing tumors to grow. Most lymphomas are made up of white blood cells called either
T cells or B cells.2
Lymphoma cells are sometimes found in the blood, but tend to form solid tumors in the
lymph system or in organs. These tumors can often be felt as a painless lump or swollen
gland almost anywhere in the body.
The lymphomas are commoner than the leukaemias and are increasing in insidence for

reasons wich are unclear.1

About the Lymphatic System
The lymphatic system helps to filter impurities, bacteria, and viruses from the body. The
lymphatic system is made up of the
lymph nodes, spleen, and special tubes that extend throughout the body like blood vessels.1
Swollen glands are actually enlarged lymph nodes. Lymph nodes act as alert centers which
activate the immune system to attack viruses, bacteria, or other foreign substances.12

Main functions of lymphatic system 9
1. To collect and return interstitial fluid, including plasma protein to the blood,
and thus help maintain fluid balance.
2. To defend the body against disease by producing lymphocytes.
3. To absorb lipids from the intestine and transport them to the blood.

Lymph nodes: Human lymph nodes are bean-shaped and range in size from a few millimeters to
about 1-2 cm in their normal state. They may become enlarged due to a tumor or infection.10
White blood cells are located within honeycomb structures of the lymph nodes. Lymph nodes are
enlarged when the body is infected due to enhanced production of some cells and division of
activated T and B cells. In some cases they may feel enlarged due to past infections; although
one may be healthy, one may still feel them residually enlarged.12

Lymphoma Causes
The exact causes of lymphoma are not known.1 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.
• Infections
o Infection with HIV
o Infection with human T-lymphocytic virus type 1 (HTLV-1)
o Infection with Epstein-Barr virus (EBV), one of the etiologic factors in mononucleosis
o Infection with Helicobacter pylori, a bacterium that lives in the digestive tract
o Infection with hepatitis B or hepatitis C virus

• Medical conditions that compromise the immune system
o HIV
o Autoimmune disease
o Diseases requiring immune suppressive therapy, often used
o following organ transplant
o Inherited immunodeficiency diseases (severe combined
o
o immunodeficiency, ataxia telangiectasia, among a host of others)11,12

• Exposure to toxic chemicals
o Farm work or an occupation with exposure to certain toxic chemicals such
o as pesticides, herbicides, or benzene and/or other solvents
o Black hair dye, which for more than 20 years has been linked to higher rates of NHL

• Genetics: Family history of lymphoma 4

What Are Grading and Staging?
When a doctor has found cancer cells and is sure that they are from a lymphoma, it is
important to know the grade and the stage of the cancer. Lymphomas of different grades
and stages grow at different rates, and respond differently to treatment5
• The grade of a lymphoma refers to how quickly, or aggressively, it isgrowing
•
• The stage of lymphoma or any cancer depends on how far it has spread throughout the body.
•
• Grade and stage are the most important factors for predicting how a patient will do and for deciding on the best treatment.5,6

Classifications of lymphoma
Lymphoma can classify to two main categories. 1
▪ Hodgkin’s lymphoma
▪ Non Hodgkin’s lymphoma

Hodgkin’s Lymphoma (Hodgkin’s Disease)
Hodgkin’s Lymphoma or Hodgkin’s Disease is a malignant growth of cells in the
lymph system. Hodgkin’s Disease is the better known form of lymphoma. It is a
rare disease. (in UK- 2.5/100 000) (2) Hodgkin’s has a long and rich history. The
disease was named after Thomas Hodgkin (1798-1866), 3,6

What now differentiates Hodgkin’s lymphoma is the presence of Reed-Sternberg
cells (and variations on this cell) in the cancerous area, a cell specific to
Hodgkin’s Lymphoma. Hodgkin’s may be more prevalent in people who have
contracted infectious mononucleosis. 6

Hodgkin’s can occur in children and adults. It is more common in two age
groups – early adulthood (ages 15-40, usually around 25-30) and late adulthood
(after 55). This lymphoma is rare in children under 5. About 10% to 15% of
cases are diagnosed in children 16 years old and younger. 12
Hodgkin’s lymphoma is not contagious and the patient does not pose a risk to
others in any way. 10

Stages of Hodgkin’s Lymphoma
*Stage I involves one lymph node region
*Stage II involves two or more lymph node regions on the same side of the diaphragm
*Stage III involves lymph nodes on both sides of the diaphragm
*Stage IV involves other organs besides the lymph system

Survival Rates by Stage 3
Stage 5-year relative survival rate
I 90% to 95%
II 90% to 95%
III 85% to 90%
IV 80% to 85%
Pathological classification of Hodgkin’s Lymphoma 5,6
◘ Classical Hodgkin’s Lymphoma
◘ Nondular sclerosis Hodgkin’s Lymphoma
◘ Nondular lymphocyte predominant Hodgkin’s Lymphoma
◘ Lympho cyterich Hodgkin’s Lymphoma
◘ Mixed cellularity Hodgkin’s Lymphoma
◘ Lymphocyte depleted Hodgkin’s Lymphoma

Nodular sclerosis- (NS)-The lymph nodes in the lower neck, chest and collarbone usually
contain normal and reactive lymphocytes and Reed-Sternberg cells separated by bands of scar-
like tissues. NS accounts for 60-70% of Hodgkin’s cases. NS appears to account for the increase
in Hodgkin’s cases in recent years 6

Nodular lymphocyte predominant- (NLP) Hodgkin’s lymphoma – while the 4 types above are
“Classical” types, NLP is in a category of its own. Typical Reed-Sternberg cells are rare to non-
existent; instead variants called L & H cells (colloquially “popcorn cells”) are seen. 12

Diagnosis of Hodgkin’s Lymphoma
There are some symptoms for Hodgkin’s but they are not specific. Often a lymph
node swells, especially in the upper body area. Other times one feels they have a lack of
energy. More serious symptoms can include weight loss, fever, and drenching night
sweats. 12
Hodgkin’s is medically diagnosed by taking a tissue sample (biopsy) and
searching for the presence of Reed-Sternberg cells, a cell specific to Hodgkin’s
lymphoma

Treatment for Hodgkin’s Lymphoma

There are treatments for all patients with Hodgkin’s lymphoma. The main types of
treatment are:
1. Chemotherapy -(using drugs to kill cancer cells and shrink tumors).
2. Bone marrow and peripheral blood transplants – transplants (actually high
dose chemotherapy and/or radiotherapy with a “rescue” of the immune
system) are being used for certain patients, especially with recurrent disease.
3. Immunotherapy is being studied in Hodgkin’s treatment including monoclonal
antibody therapy (such as rituxan) and vaccine therapy may not be far off.
4. Radiation- therapy is the use of high-energy x-rays to kill cancer cells and
shrink tumors. 4,5,6

Non-Hodgkin’s Lymphoma
Non-Hodgkin’s lymphoma is a malignant (cancerous) growth of B or T cells in the
lymph system. What causes Non-Hodgkin’s lymphoma is still unknown. Incidence
of NHL has continued to increase over the years.7 The current thinking is that
there probably is a genetic factor and the cancer may not start without a “trigger”
such as environmental factors. NHL is not contagious and the patient does not
pose a risk to others in any way. 8

Grades of Non-Hodgkin’s Lymphoma
For Non-Hodgkin’s lymphoma, grade is the most important factor in predicting the likely
outcome with and without treatment. Lymphomas are usually divided into three main
grade categories: 8
1. Low-grade or indolent: slow-growing lymphomas that can go for many years
without treatment.
2. Intermediate-grade or aggressive: faster-growing lymphomas.
3. High-grade or highly aggressive: very fast-growing lymphomas. 5,8

Different Types of Non-Hodgkin’s Lymphoma 7,8
◘ lymphoplasmacytoid lymphoma
◘ Burkitt’s lymphoma
◘ monocytoid B-cell lymphoma
◘ anaplastic large-cell lymphoma
◘ mantle cell lymphoma
◘ adult T-cell lymphoma

Lymphoblastic lymphoma (LBL) – This form occurs more often in children than adults,
and accounts for about 30% of all lymphomas in children. It is an aggressive, fast-
growing form of lymphoma.7 In the past, it has been fatal for many patients. Today, intensive
chemotherapy has greatly improved the chance of surviving LBL 12

Burkitt’s lymphoma – This form of lymphoma has been found in Africa, where infection with
Epstein-Barr virus may play a role in its cause.8 Burkitt’s lymphoma is also seen in other parts
of the world, but in most of these cases a virus doesn’t seem to be involved. Burkitt’s usually
causes a large tumor either in the bone of the jaw, or in the abdomen 7

◙ Diagnosis of Non-Hodgkin’s Lymphoma
There are some symptoms for Non-Hodgkin’s but they are not specific. Often a
lymph node swells, especially in the upper body area. Other times one feels that they
have a lack of energy. More serious symptoms can include weight loss, fever, night
sweats, or unexplained itching.7
NHL is medically diagnosed by taking a tissue sample (in a surgical procedure called a
biopsy). A needle biopsy is sometimes used but a surgical biopsy, removal of a whole
node, is preferred in getting enough tissue for a definite diagnosis. 12

1. Physical Exam 5
This includes checking the lymph nodes in the neck, armpit, or groin and
checking for an enlarged liver or spleen.

2. Blood Tests 5
The blood will be checked to see if cancer cells or cancer-related enzymes
are present. Other factors in the blood, such as anemia may be looked at.
3. Imaging Tests 6
4. Biopsy 5

Treatments for Non-Hodgkin’s Lymphoma
1. Chemotherapy and Radiation Therapy
2. Bone Marrow Transplant
3. Biological Therapy

Chemotherapy and Radiation Therapy
Chemotherapy is the use of drugs to kill cancer cells.Chemotherapy for non-Hodgkin’s
lymphoma usually includes a combination of several drugs. 8
Radiation therapy uses high-energy x-rays to kill cancer cells and shrink tumors.
Chemotherapy and radiation therapy are the most common treatments for non-Hodgkin’s
lymphoma. Because of the risk that a lymphoma has spread beyond the original tumor
surgery alone isn’t usually enough. 12
Chemotherapy is called a systemic treatment because the drugs travel throughout the
body. This means that even those cancer cells that have not yet been found may be killed.
Patients may receive chemotherapy alone or in combination with radiation therapy. 10

Bone Marrow Transplant
One form of chemotherapy called high-dose chemotherapy (HDCT), uses very high doses
of toxic drugs to kill all possible tumor cells. Because these high doses also kill most of
the bone marrow, patients are then given a bone marrow transplant to restore their ability to
make new red and white blood cells.8
Bone marrow may be taken from the patient before chemotherapy begins and given back
to the patient after treatment is done. Or, bone marrow from another person may be used. 10

Biological Therapy
Biological therapy also called biological response modifier therapy (BRMT), uses
chemicals made by the body’s own cells in order to activate the body’s defenses against
cancer. 8
The different approaches to biological therapy include:
@ Immunotherapy
@ Angiogenesis inhibitors
@ Gene therapy

Immunotherapy
In one kind of immunotherapy chemicals called cytokines are used to activate white
blood cells. Sometimes, when the immune system is activated in this way, it will fight
and kill tumor cells. Two cytokines being used are called interferon and interleukin. 8,12
*Antibodies are proteins that help white blood cells fight off viruses and bacteria.
*Antibodies bind to foreign invaders and signal the immune cells to attack 10

Angiogenesis inhibitors
Angiogenesis inhibitors are chemicals that block the formation of new blood vessels.
Tumors need to create a whole new blood supply in order to keep growing, so they cause
new blood vessels to be formed.11 In mice, angiogenesis inhibitors have blocked the
growth of many types of cancer. This highly experimental form of treatment has been
successful in mice.5 Clinical trials are underway to test the treatment in people.

Gene therapy
In gene therapy pieces of DNA are placed into cells to correct something that has gone
wrong with those cells, or to make the cells self-destruct.1 Because most cancers are now
known to result from damage to genes that keep cells from growing out of control, gene
therapy of cancer cells may someday be able to correct the problem or force cancer cells
to destroy themselves.11 Gene therapy for cancer is still highly experimental.

Non-Hodgkin’s lymphomas in Asia.

The relative frequencies of the various histopathologic types of lymphomas are generally
similar among Asian countries. Hodgkin’s disease and follicular lymphomas are relatively
rare in Asia.9 Among NHL, the Asians have a higher rate of aggressive NHL, as compared
with the NCI data. Immunologic analysis revealed that PTCL is common in Asia. The
relative frequency of PTCL is comparable among Chinese in Taiwan, the east coast of
China, and Hong Kong, as well as in adult T-cell leukemia/lymphoma (ATLL)
nonendemic areas in Japan. The increased rate of T-cell lymphomas in Asia is attributed
to the low incidence of follicular lymphomas. The similar patterns of distribution in
histopathologic and immunologic subtypes of NHL in Asia suggest that a common ethnic
or geographic factor exists.12 To elucidate it, further detailed epidemiologic studies are
needed. Primary extranodal NHL is slightly more prevalent in Asia than in the United
States; the most frequent primary site is Waldeyer’s ring in Japanese patients and the GI
tract in Chinese patients.12 Primary small intestinal lymphoma in Asia showed the pattern
of the Western type. Primary cutaneous lymphomas are rare in Asia. The clinical features
of PTCL in Asia are comparable with those described in the United States, except for a
predilection for the nasal/paranasal region. In Asia, outside Japan, ATLL has been
reported only in Taiwan. The seroepidemiologic survey of carriers of ATLL showed the
rate of seropositivity for HTLV-I in Taiwan was similar to that in nonendemic areas in
Japan. The clinicopathologic features of ATLL in Taiwan and Japan are essentially9
identical. In children in Japan and Taiwan, Hodgkin’s disease is much less frequent than
in the West. However, the relative frequencies of the histopathologic and immunologic
subtypes of childhood NHL in Japan and Taiwan do not differ significantly from those of
the West.9 Although Burkitt’s lymphoma in Japan and Taiwan is of nonendemic type, in
India it may comprise both endemic and nonendemic types in almost equal number.

Side effects of treatments
Treatments for cancer are often quite toxic. Drugs or radiation must kill cancer cells and
in the process some normal cells are also damaged. 12 This leads to the development of side-
effects. Most side-effects of treatment are only temorary. They usually resolve soon after
treatment is complete. 6 Here is a list of common side effects and how to deal with them.

1. Fall in blood counts and infections

Often during a course of chemotherapy or radiotherapy, your blood counts may fall

below normal levels. The white cells in your blood are the most commonly affected.

When white cell counts drop, your body can develop an infection and you may have a

fever. 6,8 You should contact your doctor immediately if you have fever. You may need

treatment to increase your counts and tackle infections. A fall in red blood cells or

platelets may require transfusions. 12

2. Feeling sick

Some chemotherapy drugs can make you feel sick or nauseous. You may even have a few

episodes of vomiting. This may also occur during radiotherapy to the abdomen. Most of

the time the nausea is prevented or reduced by administering drugs (called anti-emetics)

before each cycle of chemotherapy or an exposure of radiotherapy is given. 11 With

excellent new medicines available, nausea and vomiting can usually be controlled quite

effectively. 4

3. Hair loss

Hair loss is common after chemotherapy. The extent of hair loss (also called ‘alopecia’)

depends on the drugs being used in the chemotherapy. Some highly effective drugs also

cause a lot of hair loss. 7 Hair almost always starts growing back within a few months of

completing treatment, though it may take up to a year before it can grow back to normal. 4

You should discuss issues like wearing wigs or turbans with your doctor or counselor,

during and after treatment. 11

4. Sore mouth

Radiotherapy to the neck, as well as some chemotherapy drugs may cause a sore mouth a

few weeks into treatment. 4 You may feel difficulty or pain in swallowing food or drinking

fluids. Usually these symptoms are controlled well with pain medicines and temporarily

switching to mashed food that is easier to swallow. This problem is always temporary,

and reduces soon after treatment is completed. 7

5. Sore skin

Radiotherapy to the neck or chest occasionally causes a short duration of soreness on

your skin. 11 This is rarely severe because the radiotherapy doses in lymphoma are usually

low. Consult your doctor if you have this problem. Often this requires no action. If severe

enough, your doctor may stop radiation for a couple of days or change the area being

treated. 12

6. Changes in taste and appetite

Many individuals on chemotherapy or radiotherapy develop a change in taste during

treatment. Taste usually returns to normal after completing treatment. 7 Most patients

develop a loss of appetite. You may need to take small meals more frequently and drink

plenty of fluids. It is very important to maintain your nutrition during treatment. Speak to

your doctors and nurses and work out a diet that suits you best. 12

7. Fatigue

Treatment related fatigue is common, though not well understood. Any sort of cancer

treatment may leave you feeling tired.2 This is sometimes aggravated by the loss of

appetite that leads to many patients eating less than required. It is very important that you

maintain a healthy diet and eat in sufficient quantity.6 Some light exercise on each day

may also help you feel better.

Acknowledgement
●Author wishes to special thanks to Dr.Amaranath Karunanayake to help and give advices to
success this project.
●Author also wishes to thanks group members D.V.K.Wijemanne, W.Wijenayeke, G.Wijekoon
and A.S.Wijeratne to help for this project.