LYMPHOMA

The lymphatic system is a series of lymph nodes and vessels that move lymph fluid through the body. Lymph fluids contain white blood cells.

Lymphoma is a cancer of the lymphatic system. It develops in lymphocytes, which are a type of white blood cell and they help fight infections in the body and play an essential role in the body’s immune defences. Lymphocytes are in the lymph nodes, spleen, thymus, bone marrow, and other parts of the body. In lymphoma, lymphocytes change and grow uncontrollably.

As this type of cancer is present in the lymphatic system, it can quickly metastasize, or spread, to various tissues and organs throughout the body. Lymphoma most often spreads to the liver, bone marrow, or lungs.

Lymphoma can occur in any age, but it is among the most common causes of cancer in children and young adults aged 15–24 years. It is often treatable.

TYPES OF LYMPHOMAS:

There are two main types of lymphoma: Hodgkin and non-Hodgkin lymphoma. Within them, there are many subtypes.

1) Non-Hodgkin Lymphoma (NHL):

It is the most common type, typically develops from B and T lymphocytes (cells) in the lymph nodes or tissues throughout the body. It is three times more common than Hodgkin’s lymphoma and accounts for 95% of lymphoma cases. It has many subtypes which are named by the cells they affect, and if the cells are fast- or slow-growing. Non-Hodgkin Lymphoma forms in either the B-cells or T-cells of the immune system.

Most NHL types affect B-cells. Types include:

i) B-cell lymphoma:

Diffuse large B-cell lymphoma (DLBCL) is the most aggressive type of NHL. This fast-growing lymphoma originates from abnormal B cells in the blood. It is curable if treated; however, if left untreated, can lead to death. The stage of DLBCL helps determine the prognosis.

ii) T-cell lymphoma:

T-cell lymphoma is not as common as B-cell lymphoma; it accounts for only 15% of all NHL cases.

iii) Burkitt’s lymphoma:

Burkitt’s lymphoma is a rare type of NHL, is aggressive and most commonly seen in people with compromised immune systems. This type of lymphoma is most common in children in sub-Saharan Africa; however, it does occur in other parts of the world.

iv) Follicular lymphoma:

This type begins in the white blood cells and is most common in older individuals. The average age at which gets diagnosed is 60 years. This lymphoma is a slow-growing.

v) Mantle cell lymphoma:

This aggressive form of lymphoma is rare and accounts only about 6% of NHL cases. It is more commonly diagnosed at a later stage, and usually involves the gastrointestinal tract or bone marrow.

vi) Primary mediastinal B-cell lymphoma:

This subtype of B-cell lymphoma accounts for almost 10% of DLBCL cases and predominantly affects women in their 20s and 30s.

vii) Small lymphocytic lymphoma:

Small lymphatic lymphoma (SLL) is a type of slow-growing lymphoma. The cancer cells of SLL are found mostly in the lymph nodes. SLL is identical to chronic lymphocytic leukaemia (CLL), but with CLL, the majority of cancer cells are found in the blood and bone marrow.

viii) Waldenstrom macroglobulinemia (lymphoplasmacytic lymphoma):

Lymphoplasmacytic lymphoma (LPL) is a rare type of cancer and accounts for just 1 to 2 percent of all lymphomas. It mostly affects older adults. Waldenstrom macroglobulinemia is a subtype of LPL and it causes the abnormal production of antibodies.

2) Hodgkin lymphoma:

Hodgkin lymphoma typically starts in B-cells of the immune system which become abnormally large. These abnormally large B lymphocytes are called Reed-Sternberg (RS) cells. In people with Hodgkin lymphoma, cancer usually spreads from one lymph node to an adjacent one. Types of Hodgkin's lymphoma are given as follows:

i) Lymphocyte-depleted Hodgkin’s disease:

This rare, aggressive type of lymphoma accounts for only 1% of lymphoma cases, and it’s most commonly diagnosed in individuals in their 30s. The diagnostic tests will show normal lymphocytes with an abundant number of RS cells.

Patients with immuno-compromised conditions, such as HIV, are more likely to be diagnosed with this type of lymphoma.

ii) Lymphocyte-rich Hodgkin’s disease:

This type is more common in men and accounts for about 5% of Hodgkin’s lymphoma cases. It is typically diagnosed at an early stage and the diagnostic tests show the presence of both lymphocytes and RS cells.

iii) Mixed cellularity Hodgkin’s lymphoma:

This type also contains both lymphocytes and RS cells. It is more common —accounts for almost 25% of Hodgkin’s lymphoma cases and it’s more prevalent in older adult men.

iv) Nodular lymphocyte-predominant Hodgkin’s disease:

Nodular lymphocyte-predominant Hodgkin’s disease (NLPHL) type of Hodgkin’s lymphoma occurs in about 5% of lymphoma cases and is characterized by an absence of RS cells.

It is most common in 30 and 50 years of age, and it’s more common in males. The chance of NLPHL progressing or transforming into a type of aggressive NHL is rare.

v) Nodular sclerosis Hodgkin’s lymphoma:

It is a common type and occurs in 70% of Hodgkin’s lymphoma cases, and is more common in young adults than any other age group. This type of lymphoma occurs in lymph nodes that contain scar tissue, or sclerosis.

This type of lymphoma is highly treatable with a high cure rate.

LYMPHOMA CAUSES:

It is not known what causes lymphoma in most cases. However, there may be some risk factors.

RISK FACTORS FOR LYMPHOMA:

Both types of lymphomas have different risk factors:

1) Non-Hodgkin lymphoma:

Risk factors for non-Hodgkin lymphoma include:

Age: Lymphomas are most common in people aged 60 years or more. However, some types are more common in children and young adults.

Sex: Some types are more likely in women. Men have a higher risk of other types.

Ethnicity and location: In the United States, African American and Asian American people are at a lower risk for non-Hodgkin's lymphoma than white people. Non-Hodgkin's Lymphoma is more common in developed nations.

Exposure to chemicals and radiation: Nuclear radiation and certain agricultural chemicals like pesticides, fertilizers and herbicides increase the risk of non-Hodgkin's lymphoma.

Immunodeficiency: People with a weak immune system and those taking an immune system-suppressing drug after an organ transplant are at higher risk.

Autoimmune diseases: These are the diseases in which the immune system attacks the body’s own cells. Examples include rheumatoid arthritis and celiac disease.

Infection: People who have had infections such as the human T-cell leukaemia/lymphotropic virus (HTLV-1), Heliobacter pylori, hepatitis C, or the Epstein-Barr virus (EBV) are associated with an increased risk.

Breast implants: These can lead to anaplastic large cell lymphoma in the breast tissue.

Bodyweight and diet: Obesity is a possible risk factor for lymphoma, but more research is needed to prove that.

2) Hodgkin lymphoma:

Risk factors for Hodgkin lymphoma include:

Age: People in the age group of 20–30 years and those over 55 years of age have a higher risk of lymphoma.

Sex: Hodgkin lymphoma is slightly more common in males than females.

Family history: If a sibling has Hodgkin lymphoma, the risk is slightly higher. If the sibling is an identical twin, this risk increases significantly.

Infectious mononucleosis: The Epstein-Barr virus (EBV) can cause infectious mononucleosis which increases the risk of lymphoma.

Immunodeficiency: Individuals with HIV are at a higher risk of developing lymphoma.

SYMPTOMS OF LYMPHOMA:

The symptoms of lymphoma are similar to some viral diseases, such as the common cold. However, they typically continue for a more extended duration.

Sometimes, people do not experience any symptoms. Some may notice swelling of lymph nodes in the neck, upper chest, groin, abdomen or armpits.

The swellings are often painless. They may become painful if the enlarged glands press on organs, bones, and other structures.

Lymph nodes swelling can be seen in common infections, such as a cold. In lymphoma, the swelling does not go away. The swelling becomes painful if it is due to an infection.

If any persistent swelling is observed by a person, he/she should seek medical consultation.

Other symptoms which may be seen in both types of lymphoma include:

· persistent fever without any infection

· night sweats and chills

· unexplained weight loss and reduced appetite

· unusual itching

· persistent fatigue or a lack of energy

· pain in lymph nodes after drinking alcohol

Some additional symptoms of non-Hodgkin lymphoma include:

· persistent coughing

· shortness of breath

· pain or swelling in the abdomen

Sometimes, if an enlarged lymph node presses against spinal nerves or the spinal cord; it may cause pain, weakness, paralysis, or altered sensation.

STAGES OF LYMPHOMA:

Both non-Hodgkin's lymphoma and Hodgkin’s lymphoma can be classified into four stages. The stage of lymphoma is determined by the location of cancer, type, growth rate, and cellular characteristics and its spread.

· Stage 1: Cancer is in one lymph node or one organ.

· Stage 2: Cancer is in two adjacent lymph nodes and on the same side of the body (ipsilateral), or the cancer is in one organ and nearby lymph nodes.

· Stage 3: Cancer is in lymph nodes on both sides of the body (bilateral) and multiple lymph nodes.

· Stage 4: Cancer can be in an organ and spread beyond nearby lymph nodes. As non-Hodgkin's lymphoma progresses, its spread may start. The most common sites for advanced non-Hodgkin's lymphoma include the liver, bone marrow, and lungs.

While stage 4 lymphoma is advanced, it can still be treated.

DIAGNOSIS:

The doctor will ask for a detailed history to the patient including questions about any pain, infection, swelling, fever, any weaknesses, appetite changes, weight changes; also past medical history and family history and try to rule out other conditions.

Tests and procedures used to diagnose lymphoma include:

Physical examination:

Doctor will do a physical exam, including an inspection of the abdomen and chin, neck, groin, and armpits, where lymph node swellings may occur. They may also check for swollen spleen or liver.

The doctor will look for signs of infection near lymph nodes since it can be the reason for most cases of swelling.

Lymph node biopsy:

In this procedure, the surgeon will remove all or a section of a lymph node, or use a needle to take a small amount of tissue from the affected node and send it to the laboratory. Advanced tests can determine if lymphoma cells are present and what types of cells are involved.

Blood tests:

The number of certain cells, levels of other substances, or evidence of infection in your blood can help the doctor to arrive at a diagnosis.

Removing a sample of bone marrow for testing:

This procedure involves inserting a needle into your hipbone to remove a sample of fluid or tissue from bone marrow. The sample is analyzed to look for lymphoma cells.

Molecular test:

This test is used to find changes to genes, proteins, and other substances in cancer cells which helps the doctor to figure out the type of lymphoma.

Imaging tests:

Doctor may recommend imaging tests to look for signs of lymphoma in other areas of the body. Tests may include:

· Computed Tomography (CT) scan

· Chest X-ray: Done using low doses of radiation to make images of the inside of the chest.

· X-ray imaging of the abdomen and pelvis.

· Magnetic Resonance Imaging (MRI): Powerful magnets and radio waves are used to make pictures of organs and structures inside the body.

· Positron Emission Tomography (PET) scan: In this test, a radioactive substance is used to look for cancer cells in the body.

· Ultrasound.

TREATMENT:

The treatment plan depends on various factors such as the type and stage of the disease, overall health, and patient's preferences.

Various treatment options include:

Active surveillance:

Some forms of lymphoma are very slow-growing. Doctor may decide to wait to treat the lymphoma when it causes signs and symptoms that interfere with the daily activities. Until then, periodic tests may be done to monitor the condition.

Chemotherapy:

Chemotherapy includes the use of drugs to destroy fast-growing cells, such as cancer cells. The drugs are usually administered through a vein or given in form of a pill.

Radiation therapy:

This type of therapy is recommended to target and destroy small areas of cancer. High-powered beams of energy, such as X-rays and protons are used to kill cancer cells.

Bone marrow transplant:

A bone marrow transplant, also known as a stem cell transplant helps restore damaged bone marrow following high dose chemotherapy or radiation therapy.

Two types of stem cell transplants can be done:

· An autologous transplant uses your own stem cells.

· An allogeneic transplant uses stem cells taken from a donor.

Surgery:

A surgeon may remove the spleen or other organs after the lymphoma has spread.

Biologic therapy/ Immunotherapy:

This treatment stimulates the immune system to attack cancer.

Antibody therapy:

Synthetic antibodies are injected into the bloodstream which response to cancer’s toxins.

Radioimmunotherapy:

High powered radioactive doses are delivered directly into cancerous B cells and T-cells to destroy them.

Steroids:

Steroids are injected to treat lymphoma.

Other treatments:

Chimeric antigen receptor (CAR)-T cell therapy is a specialized treatment which takes the body's germ-fighting T cells, builds them to fight cancer and infuses them back into your body.

The main treatments for non-Hodgkin lymphoma are:

· Chemotherapy

· Radiation therapy

· Immunotherapy

· Targeted therapy

The main treatments for Hodgkin lymphoma are:

· Chemotherapy

· Radiation therapy

· Immunotherapy

Various medical specialists need to collaborate to collectively plan the treatment of lymphoma. Haematologists are the doctors who specialize in blood, bone marrow, and immune cell disorders. Oncologists are the ones who treat cancerous tumours. Pathologists work with these doctors to assist in treatment planning and identify if a particular treatment is working or not.

An excellent team of specialists like that of haematologists, oncologists, pathologists and surgeons can be found at the Kingsway Hospitals, Nagpur.