Introduction

Hodgkin’s lymphoma is a form of cancer that begins in cells of the immune system.

The immune system fights infections and other diseases. Hodgkin’s lymphoma happens when cells in the immune system become abnormal and begin to make copies of themselves in an uncontrollable way.

Hodgkin’s lymphoma is highly treatable. Most people diagnosed with Hodgkin’s lymphoma can now be cured, or their disease can be controlled for many years.

This reference summary explains what Hodgkin’s lymphoma is, as well as its causes, symptoms and treatment options.

Cancer and Its Causes

Hodgkin’s disease is a type of lymphoma. Lymphomas are cancers that develop in the lymphatic system, from the B type of immune system cells.

Cells contain hereditary, or genetic, materials called chromosomes. Chromosomes control the growth of cells.

Normal cells in the body grow and die in a controlled way. When the chromosomes in a cell become abnormal, the cell can lose the ability to control its growth.

In Hodgkin’s lymphoma, cells in the lymphatic system begin growing abnormally.

In most cases of Hodgkin’s lymphoma the abnormal cells are called Reed-Sternberg cells.

The new abnormal cells divide again and again, making more and more abnormal cells. The abnormal cells don't die when they should. They don't protect the body from infections or other diseases. The buildup of extra cells often forms a mass of

tissue called a growth or tumor. Sudden changes in genetic material can happen for a variety of reasons.

These changes are sometimes inherited. Changes in chromosomes may also occur as a result of exposure to infections, drugs, tobacco, chemicals, or other factors.

Other risk factors for Hodgkin’s lymphoma include:

• Age

• A family history of lymphoma

• Gender: males are at a higher risk than females

• Past infections with the Epstein Bar virus and a weakened immune system.

There are two main types of Hodgkin’s lymphoma:

    • classical Hodgkin’s lymphoma and nodular lymphocyte-predominant
    • Hodgkin’s lymphoma.

Most people with Hodgkin’s lymphoma have the classical type. Classical Hodgkin’s lymphoma begins when a lymphocyte, usually a B cell, becomes abnormal.

Nodular lymphocyte-predominant Hodgkin’s lymphoma is a rare type of Hodgkin’s lymphoma. The abnormal cells are called “popcorn cells”. It may be treated differently from the classical type.

Because lymphatic tissue is in many parts of the body, Hodgkin’s lymphoma can start almost anywhere. Usually, it's first found in a lymph node above the diaphragm, the thin muscle that separates the chest from the abdomen.

But Hodgkin’s lymphoma also may be found in a group of lymph nodes. Sometimes it starts in other parts of the lymphatic system.

Signs and Symptoms

The most common symptom of Hodgkin’s Lymphoma is a painless swelling in the lymph nodes in the neck, underarm or groin.

Other common symptoms may include:

• Fever and chills

• Severe night sweats

• Weakness and tiredness

• Unexplained weight loss

• Trouble breathing, coughing or chest pain

Other symptoms include:

• Becoming more sensitive to the effects of alcohol or having painful lymph nodes after drinking alcohol

• Persistent fatigue

• Itching

These symptoms may not be caused by Hodgkin’s lymphoma. Common problems like infections or the flu can cause similar symptoms. Make sure to see a doctor if your symptoms last more than two weeks.

Diagnosis

If you have swollen lymph nodes or another symptom that suggests Hodgkin’s lymphoma, your doctor will try to find out what is causing the problem. Your doctor may ask about your personal and family medical history.

Your doctor will check for swollen lymph nodes in your neck, underarms, and groin.

Your doctor may also check for a swollen spleen or liver.

Your doctor may order a CBC, also known as a complete blood count test. This gives information about the quantity and quality of each type of cell in your blood.

Your doctor may order other tests such as a CT scan, MRI scan, PET scan, x-rays and a lymph node biopsy.

Chest x-rays may be ordered to look for swollen lymph nodes or other signs of lymphoma in your chest. This is done to see how far the lymphoma has spread. It is known as staging.

CT stands for Computed Tomography. A specialized x-ray machine linked to a computer takes a series of detailed pictures of your chest, abdomen, and pelvis. You may receive an injection of contrast material.

Also, you may be asked to drink another type of contrast material. The contrast material makes it easier for your doctor to see swollen lymph nodes and other abnormal areas on the scan.

MRI stands for Magnetic Resonance Imaging. A powerful magnet linked to a computer is used to make detailed pictures of your bones, brain, or other tissues. Your doctor can view these pictures on a monitor and can print them on film.

During a PET scan you receive an injection of a small amount of radioactive sugar. A machine makes computerized pictures of the sugar being used by cells in your body. Lymphoma cells use sugar faster than normal cells and areas with lymphoma look brighter on the pictures.

A biopsy is the only sure way to diagnose Hodgkin’s lymphoma. There are several different kinds of biopsies.

In a fine needle aspiration, a needle may be inserted in a lymph node, and fluid and tissue samples taken for pathological studies. Usually this kind of biopsy cannot get a large enough sample to diagnose Hodgkin’s lymphoma.

In an incisional biopsy, a small part of a lymph node is taken out through an incision.

In an excisional biopsy, an entire lymph node and some surrounding tissue is removed through an incision. This type of biopsy is the best at getting a good enough sample for diagnosis. The pathologist uses a microscope to check the tissue for Hodgkin’s lymphoma cells.

A person with Hodgkin’s lymphoma usually has large, abnormal cells known as Reed- Sternberg cells. They are not found in people with non-Hodgkin’s lymphoma.

Staging

A stage is an indication of how widely the disease has spread.

Staging involves several procedures to determine what parts of the body are affected by the disease. Treatment can be recommended based on the stage of the cancer.

Hodgkin’s lymphoma tends to spread from one group of lymph nodes to the next. For example, Hodgkin’s lymphoma that starts in the lymph nodes in the neck may spread first to the lymph nodes above the collarbones, and then to the lymph nodes under the arms and within the chest.

In time, the Hodgkin’s lymphoma cells can invade blood vessels and spread to almost any other part of the body. For example, it can spread to the liver, lungs, bone, and bone marrow.

Staging can involve one or more of the following tests: CT scans, MRIs, PET scans and a bone marrow biopsy. A thick needle is used to remove a small sample of bone and bone marrow from your hipbone or another large bone. Local anesthesia can help control pain.

A pathologist looks for Hodgkin’s lymphoma cells in the sample. Your doctor may also recommend biopsies of the other lymph nodes, the liver or other tissue.

The stage is decided based on the number of lymph nodes that have Hodgkin’s lymphoma cells, if these lymph nodes are on one or both sides of the diaphragm, and if the disease has spread to the bone marrow, spleen, liver, or lung. The diaphragm is a large, thin muscle that divides the chest from the abdomen. It is very important in breathing.

Stages are usually described using the numbers 1 - 4; a lower number indicates an earlier stage.

Stage 1: The lymphoma cells are in one lymph node group (such as in the neck or underarm). Or, if the lymphoma cells are not in the lymph nodes, they are in only one part of a tissue or an organ (such as the lung).

Stage 2: The lymphoma cells are in at least two lymph node groups on the same side of (either above or below) the diaphragm. Or, the lymphoma cells are in one part of a tissue or an organ and the lymph nodes near that organ (on the same side of the diaphragm). There may be lymphoma cells in other lymph node groups on the same side of the diaphragm.

Stage 3: The lymphoma cells are in lymph nodes above and below the diaphragm. Lymphoma also may be found in one part of a tissue or an organ (such as the liver, lung, or bone) near these lymph node groups. It may also be found in the spleen.

Stage 4: Lymphoma cells are found in several parts of one or more organs or tissues. Or, the lymphoma is in an organ (such as the liver, lung, or bone) and in distant lymph nodes.

The disease is considered recurrent if it returns after treatment. In addition to these stage numbers, your doctor may also describe A or B symptoms:

• A: You have not had weight loss, drenching night sweats, or fevers.

• B: You have had weight loss, drenching night sweats, or fevers.

Patients with B symptoms have a worse prognosis than patients with A symptoms

Treatment

Treatment for Hodgkin’s lymphoma aims at killing as many abnormal cells as possible.

Your options for treatment depend on many different factors. Your doctor will discuss your treatment options with you.

What treatments are right for you depend on:

• Your type of Hodgkin’s lymphoma (most people have classical Hodgkin’s lymphoma)

• Its stage (where the lymphoma is found)

• If you have a tumor that is more than 4 inches (10 centimeters) wide

• Your age

• Whether you've had weight loss, drenching night sweats, or fevers.

Treatment for Hodgkin’s lymphoma usually includes radiation therapy or

chemotherapy. Sometimes both are given. Treatment decisions are made depending on the stage of the disease, its location in the body, which symptoms are present and the general health and age of the patient.

For early stages of Hodgkin’s lymphoma, radiation therapy may be used. It, however, is usually used after chemotherapy.

Proton Therapy for Hodgkin’s lymphoma Treatment

Although most patients with Hodgkin lymphoma are cured, they develop many treatment side effects related to both chemotherapy and radiation.

The Childhood Cancer Survivor Study identified Hodgkin lymphoma survivors as one of the groups at highest risk for developing severe or life-threatening chronic health conditions, specifically a subsequent cancer or heart disease. In fact, the two leading causes of death more than 15 years after completing treatment in Hodgkin lymphoma survivors are second cancers and heart disease.

These increased risks in second cancers and heart disease are likely due to the late effects from chemotherapy (specifically bleomycin and adriamycin) as well as from radiotherapy.

Since proton therapy is a more precise method of radiation therapy, it is likely to cause much less heart disease and fewer second cancers than conventional radiation therapy.

The Picture shows an axial CT scan slice through the heart (red) of a patient with Hodgkin lymphoma involving the mediastinum.

On the left is the X-ray plan and on the right is the proton plan. The dark blue line in both represents the tumor and target area for the radiation.

The green line represents the volume of the body receiving 95 percent of the total prescribed dose of radiation, while the light blue line reflects the volume of the body receiving 10 percent of the total prescribed radiation dose.

As is apparent, with conventional radiation (left), the X-rays deposit more radiation in the heart and breasts than the proton plan. In fact, the proton plan reduced the mean dose to the heart by more than 50 percent and the mean dose to the breast by 70 percent.

For this reason, it’s believed that Hodgkin lymphoma patients will have a much lower risk of heart disease and second malignancy with proton therapy than what’s been observed in the past with conventional radiation therapy.

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What to expect

Each patient’s therapy is precisely tailored to their needs. Patients and clinicians collaborate closely to create a personalized treatment plan. The number and length of treatments will vary, based on the cancer. How patients respond depends on many factors, including the types of treatments they are receiving. Many people tolerate proton therapy well and continue to perform normal activities. However, individual responses vary.

Advantages of Proton Therapy for Hodgkin's Lymphoma

Although proton radiation treatment is relatively new, clinical trials for Hodgkin's Lymphoma have already shown excellent signs of disease control and minimal side effects compared with traditional forms of treatment. Proton therapy also offers a number of other compelling benefits:

    • Treatment is noninvasive and painless
    • Proton therapy is effective for treating early stage Hodgkin’s lymphoma
    • Treatment offers quicker recovery times with minimal side effects
    • It is more accurate and precise than other kinds of radiation
    • Treatment is provided in an outpatient setting
    • Proton radiation has little to no impact on patient energy level