Key Points for This Section

Childhood Hodgkin lymphoma is a disease in which malignant (cancer) cells form in the lymph system. There are two types of childhood Hodgkin lymphoma. Age, gender, and Epstein-Barr virus infection can affect the risk of developing childhood Hodgkin lymphoma. Possible signs of childhood Hodgkin lymphoma include swollen lymph nodes, fever, night sweats, and weight loss. Tests that examine the lymph system are used to detect (find) and diagnose childhood Hodgkin lymphoma. Certain factors affect prognosis (chance of recovery) and treatment options.

Childhood Hodgkin lymphoma is a disease in which malignant (cancer) cells form in the lymph system.

Childhood Hodgkin lymphoma is a type of cancer that develops in the lymph system, part of the body's immune system. The lymph system is made up of the following:

• Lymph: Colorless, watery fluid that travels through the lymph system and carries white blood cells called lymphocytes. Lymphocytes protect the body against infections and the growth of tumors.
• Lymph vessels: A network of thin tubes that collect lymph from different parts of the body and return it to the bloodstream.
• Lymph nodes: Small, bean-shaped structures that filter lymph and store white blood cells that help fight infection and disease. Lymph nodes are located along the network of lymph vessels found throughout the body. Clusters of lymph nodes are found in the underarm, pelvis, neck, abdomen, and groin.
• Spleen: An organ that makes lymphocytes, filters the blood, stores blood cells, and destroys old blood cells. The spleen is on the left side of the abdomen near the stomach.
• Thymus: An organ in which lymphocytes grow and multiply. The thymus is in the chest behind the breastbone.
• Tonsils: Two small masses of lymph tissue at the back of the throat. The tonsils make lymphocytes.
• Bone marrow: The soft, spongy tissue in the center of large bones. Bone marrow makes white blood cells, red blood cells, and platelets.

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Anatomy of the lymph system, showing the lymph vessels and lymph organs including lymph nodes, tonsils, thymus, spleen, and bone marrow. Lymph (clear fluid) and lymphocytes travel through the lymph vessels and into the lymph nodes where the lymphocytes destroy harmful substances. The lymph enters the blood through a large vein near the heart.

Because lymph tissue is found throughout the body, Hodgkin lymphoma can start in almost any part of the body and spread to almost any tissue or organ in the body.

Lymphomas are divided into two general types: Hodgkin lymphoma and non-Hodgkin lymphoma. (See the PDQ summary on Childhood Non-Hodgkin Lymphoma Treatment ¹ for more information.)

Hodgkin lymphoma can occur in both children and adults; however, treatment for children may be different than treatment for adults. (See the PDQ summary on Adult Hodgkin Lymphoma Treatment ² for more information.)

There are two types of childhood Hodgkin lymphoma.

The two types of childhood Hodgkin lymphoma are:

• Classical Hodgkin lymphoma.
• Nodular lymphocyte-predominant Hodgkin lymphoma.

Classical Hodgkin lymphoma is divided into four subtypes, based on how the cancer cells look under a microscope:

• Lymphocyte-rich classical Hodgkin lymphoma.
• Nodular sclerosis Hodgkin lymphoma.
• Mixed cellularity Hodgkin lymphoma.
• Lymphocyte-depleted Hodgkin lymphoma.

Age, gender, and Epstein-Barr virus infection can affect the risk of developing childhood Hodgkin lymphoma.

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. People who think they may be at risk should discuss this with their doctor. Risk factors for childhood Hodgkin lymphoma include the following:

• Being between the ages of 15 and 19. At these ages, Hodgkin lymphoma is slightly more common in girls than in boys. In children younger than 5 years, it is more common in boys than in girls.
• Being infected with the Epstein-Barr virus.
• Having a brother or sister with Hodgkin lymphoma.

Possible signs of childhood Hodgkin lymphoma include swollen lymph nodes, fever, night sweats, and weight loss.

These and other symptoms may be caused by childhood Hodgkin lymphoma or by other conditions. A doctor should be consulted if any of the following problems occur:

• Painless, swollen lymph nodes in the neck, chest, underarm, or groin.
• Fever.
• Night sweats.
• Weight loss for no known reason.
• Itchy skin.

Tests that examine the lymph system are used to detect (find) and diagnose childhood Hodgkin lymphoma.

The following tests and procedures may be used:

• Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken.
• Lymph node biopsy: The removal of all or part of a lymph node. One of the following types of biopsies may be done:
  • Excisional biopsy: The removal of an entire lymph node.
  • Incisional biopsy: The removal of part of a lymph node.
  • Core biopsy: The removal of tissue from a lymph node using a wide needle.
  • Fine-needle aspiration (FNA) biopsy: The removal of tissue from a lymph node using a thin needle.

  A pathologist views the tissue under a microscope to look for cancer cells, especially Reed-Sternberg cells. Reed-Sternberg cells are common in classical Hodgkin lymphoma.

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  Reed-Sternberg cell. Reed-Sternberg cells are large, abnormal lymphocytes that may contain more than one nucleus. These cells are found in Hodgkin lymphoma.

• Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
• Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
  • The number of red blood cells, white blood cells, and platelets.
  • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
  • The portion of the blood sample made up of red blood cells.

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  Complete blood count (CBC). Blood is collected by inserting a needle into a vein and allowing the blood to flow into a tube. The blood sample is sent to the laboratory and the red blood cells, white blood cells, and platelets are counted. The CBC is used to test for, diagnose, and monitor many different conditions.

• Sedimentation rate: A procedure in which a sample of blood is drawn and checked for the rate at which the red blood cells settle to the bottom of the test tube.
• Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it.
• PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
• Immunophenotyping: A test in which the cells in a sample of blood or bone marrow are looked at under a microscope to find out the type of malignant (cancerous) lymphocytes that are causing the lymphoma.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:

• The stage of the cancer.
• The size of the tumor and how quickly it shrinks after initial treatment.
• The patient's symptoms when diagnosed.
• Certain features of the cancer cells.
• Whether the cancer is newly diagnosed, does not respond to initial treatment, or has recurred (come back).

The treatment options also depend on:

• The child's age and gender.
• The risk of long-term side effects.

Most children and adolescents with newly diagnosed Hodgkin lymphoma can be cured.

Glossary Terms

The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.

The removal of cells or tissues for examination by a pathologist. The pathologist may study the tissue under a microscope or perform other tests on the cells or tissue. There are many different types of biopsy procedures. The most common types include: (1) incisional biopsy, in which only a sample of tissue is removed; (2) excisional biopsy, in which an entire lump or suspicious area is removed; and (3) needle biopsy, in which a sample of tissue or fluid is removed with a needle. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.

A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.

A procedure in which a sample of blood is examined to measure the amounts of certain substances made in the body. An abnormal amount of a substance can be a sign of disease in the organ or tissue that produces it.

The soft, sponge-like tissue in the center of most bones. It produces white blood cells, red blood cells, and platelets.

A term for diseases in which abnormal cells divide without control and can invade nearby tissues. Cancer cells can also spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord.

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

An x-ray of the structures inside the chest. An x-ray is a type of high-energy radiation that can go through the body and onto film, making pictures of areas inside the chest, which can be used to diagnose disease.

The most common type of Hodgkin lymphoma, which is a cancer of the immune system. Classical Hodgkin lymphoma is marked by the presence of a type of cell called the Reed-Sternberg cell.

A test to check the number of red blood cells, white blood cells, and platelets in a sample of blood. Also called blood cell count and CBC.

A dye or other substance that helps show abnormal areas inside the body. It is given by injection into a vein, by enema, or by mouth. Contrast material may be used with x-rays, CT scans, MRI, or other imaging tests.

The removal of a tissue sample with a wide needle for examination under a microscope. Also called core needle biopsy.

A series of detailed pictures of areas inside the body taken from different angles. The pictures are created by a computer linked to an x-ray machine. Also called CAT scan, computed tomography scan, computerized axial tomography scan, and computerized tomography.

The process of identifying a disease, such as cancer, from its signs and symptoms.

A common virus that remains dormant in most people. It causes infectious mononucleosis and has been associated with certain cancers, including Burkitt lymphoma, immunoblastic lymphoma, and nasopharyngeal carcinoma. Also called EBV.

A surgical procedure in which an entire lump or suspicious area is removed for diagnosis. The tissue is then examined under a microscope.

An increase in body temperature above normal (98.6 degrees F), usually caused by disease.

The removal of tissue or fluid with a thin needle for examination under a microscope. Also called FNA biopsy.

A type of sugar; the chief source of energy for living organisms.

The area where the thigh meets the abdomen.

The substance inside red blood cells that binds to oxygen in the lungs and carries it to the tissues.

A cancer of the immune system that is marked by the presence of a type of cell called the Reed-Sternberg cell. The two major types of Hodgkin lymphoma are classical Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma. Symptoms include the painless enlargement of lymph nodes, spleen, or other immune tissue. Other symptoms include fever, weight loss, fatigue, or night sweats. Also called Hodgkin disease.

The complex group of organs and cells that defends the body against infections and other diseases.

A process used to identify cells, based on the types of antigens or markers on the surface of the cell. This process is used to diagnose specific types of leukemia and lymphoma by comparing the cancer cells to normal cells of the immune system.

A surgical procedure in which a portion of a lump or suspicious area is removed for diagnosis. The tissue is then examined under a microscope to check for signs of disease.

Invasion and multiplication of germs in the body. Infections can occur in any part of the body and can spread throughout the body. The germs may be bacteria, viruses, yeast, or fungi. They can cause a fever and other problems, depending on where the infection occurs. When the body’s natural defense system is strong, it can often fight the germs and prevent infection. Some cancer treatments can weaken the natural defense system.

Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."

The clear fluid that travels through the lymphatic system and carries cells that help fight infections and other diseases. Also called lymphatic fluid.

A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph nodes filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels. Also called lymph gland.

A thin tube that carries lymph (lymphatic fluid) and white blood cells through the lymphatic system. Also called lymphatic vessel.

The tissues and organs that produce, store, and carry white blood cells that fight infections and other diseases. This system includes the bone marrow, spleen, thymus, lymph nodes, and lymphatic vessels (a network of thin tubes that carry lymph and white blood cells). Lymphatic vessels branch, like blood vessels, into all the tissues of the body.

A type of immune cell that is made in the bone marrow and is found in the blood and in lymph tissue. The two main types of lymphocytes are B lymphocytes and T lymphocytes. B lymphocytes make antibodies, and T lymphocytes help kill tumor cells and help control immune responses. A lymphocyte is a type of white blood cell.

Cancer that begins in cells of the immune system. There are two basic categories of lymphomas. One kind is Hodgkin lymphoma, which is marked by the presence of a type of cell called the Reed-Sternberg cell. The other category is non-Hodgkin lymphomas, which includes a large, diverse group of cancers of immune system cells. Non-Hodgkin lymphomas can be further divided into cancers that have an indolent (slow-growing) course and those that have an aggressive (fast-growing) course. These subtypes behave and respond to treatment differently. Both Hodgkin and non-Hodgkin lymphomas can occur in children and adults, and prognosis and treatment depend on the stage and the type of cancer.

Cancerous. Malignant tumors can invade and destroy nearby tissue and spread to other parts of the body.

A rare type of Hodgkin lymphoma, which is a cancer of the immune system. It is marked by the presence of a type of cell called a popcorn cell, which is different from the typical Reed-Sternberg cell found in classical Hodgkin lymphoma. This type of Hodgkin lymphoma may change into diffuse large B-cell lymphoma. Also called NLPHL.

A part of the body that performs a specific function. For example, the heart is an organ.

A colorless, odorless gas. It is needed for animal and plant life. Oxygen that is breathed in enters the blood from the lungs and travels to the tissues.

A doctor who identifies diseases by studying cells and tissues under a microscope.

PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.

The lower part of the abdomen, located between the hip bones.

A procedure in which a small amount of radioactive glucose (sugar) is injected into a vein, and a scanner is used to make detailed, computerized pictures of areas inside the body where the glucose is used. Because cancer cells often use more glucose than normal cells, the pictures can be used to find cancer cells in the body. Also called positron emission tomography scan.

An exam of the body to check for general signs of disease.

A tiny piece of a cell found in the blood that breaks off from a large cell found in the bone marrow. Platelets help wounds heal and prevent bleeding by forming blood clots. Also called thrombocyte.

The likely outcome or course of a disease; the chance of recovery or recurrence.

A molecule made up of amino acids that are needed for the body to function properly. Proteins are the basis of body structures such as skin and hair and of substances such as enzymes, cytokines, and antibodies.

Giving off radiation.

Cancer that has recurred (come back), usually after a period of time during which the cancer could not be detected. The cancer may come back to the same place as the original (primary) tumor or to another place in the body. Also called recurrence.

A cell that carries oxygen to all parts of the body. Also called erythrocyte and RBC.

A type of cell that appears in people with Hodgkin disease. The number of these cells increases as the disease advances.

Something that may increase the chance of developing a disease. Some examples of risk factors for cancer include age, a family history of certain cancers, use of tobacco products, certain eating habits, obesity, lack of exercise, exposure to radiation or other cancer-causing agents, and certain genetic changes.

In medicine, an instrument that takes pictures of the inside of the body.

The distance red blood cells travel in one hour in a sample of blood as they settle to the bottom of a test tube. The sedimentation rate is increased in inflammation, infection, cancer, rheumatic diseases, and diseases of the blood and bone marrow. Also called erythrocyte sedimentation rate and ESR.

A problem that occurs when treatment affects healthy tissues or organs. Some common side effects of cancer treatment are fatigue, pain, nausea, vomiting, decreased blood cell counts, hair loss, and mouth sores.

An organ that is part of the lymphatic system. The spleen makes lymphocytes, filters the blood, stores blood cells, and destroys old blood cells. It is located on the left side of the abdomen near the stomach.

The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.

An organ that is part of the digestive system. The stomach helps digest food by mixing it with digestive juices and churning it into a thin liquid.

An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.

An organ that is part of the lymphatic system, in which T lymphocytes grow and multiply. The thymus is in the chest behind the breastbone.

A group or layer of cells that work together to perform a specific function.

One of two small masses of lymphoid tissue on either side of the throat.

An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancer), or malignant (cancer). Also called neoplasm.

A blood vessel that carries blood to the heart from tissues and organs in the body.

A type of immune cell. Most white blood cells are made in the bone marrow and are found in the blood and lymph tissue. White blood cells help the body fight infections and other diseases. Granulocytes, monocytes, and lymphocytes are white blood cells. Also called leukocyte and WBC.

A type of high-energy radiation. In low doses, x-rays are used to diagnose diseases by making pictures of the inside of the body. In high doses, x-rays are used to treat cancer.