Lymphoma - Hodgkin - Childhood

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What is Lymphoma - Hodgkin - Childhood ?

Hodgkin lymphoma, previously called Hodgkin’s disease, is one type of lymphoma. Lymphoma is a cancer of the lymphatic or lymph node system. Lymphoma begins when cells in the lymphatic system change and grow uncontrollably, which may form a tumor.

About the lymphatic system

The lymphatic system is made up of thin tubes that branch out to all parts of the body. Its job is to fight infection and other diseases. The lymphatic system carries lymph, a colorless fluid containing lymphocytes (white blood cells). Lymphocytes fight germs in the body. B-lymphocytes (also called B cells) make antibodies to fight bacteria, and T-lymphocytes (also called T cells) kill viruses and foreign cells and trigger the B cells to make antibodies.

Groups of bean-shaped organs called lymph nodes are located throughout the body at different areas in the lymphatic system. Lymph nodes are found in clusters in the abdomen, groin, pelvis, underarms, and neck. Other parts of the lymphatic system include the spleen, which makes lymphocytes and filters blood; the thymus, an organ under the breastbone; and the tonsils, which are located in the throat.

Hodgkin lymphoma most commonly affects lymph nodes in the neck or the area between the lungs and behind the breastbone (called the mediastinum). It can also begin in groups of lymph nodes under the arms, in the groin, or in the abdomen or pelvis.

If Hodgkin lymphoma spreads, it may spread along the lymphatic channels to other lymph nodes and the spleen, or outside of the lymphatic system, most commonly to the lungs, liver, bone marrow, or bone.

Types of Hodgkin lymphoma

It is important to know the type of Hodgkin lymphoma, as this may affect the patient’s treatment plan. Doctors determine the type of Hodgkin lymphoma based on how the cells in a tissue sample look under a microscope and whether the cells contain abnormal patterns of certain proteins. There are two main types of Hodgkin lymphoma: nodular lymphocyte predominant and classical.

Nodular lymphocyte predominant Hodgkin lymphoma. This is more common among male and younger patients. The disease is usually found in the neck, underarm, or groin.

Classical Hodgkin lymphoma. There are four subtypes of classical Hodgkin lymphoma: nodular sclerosing, mixed cellularity, lymphocyte rich, and lymphocyte depleted.

Nodular sclerosis Hodgkin lymphoma. This is the most common type of Hodgkin lymphoma in adolescents and young adults in the United States and in other developed countries. Tumors are often very bulky; they most often begin in the lymph nodes in the neck, chest, or abdomen and may spread to the lungs.

Mixed cellularity Hodgkin lymphoma. This type is more common in children age 10 years or younger and shows the strongest link to the Epstein-Barr virus (the virus that causes mononucleosis). It usually begins in the lymph nodes in the abdomen or in the spleen.

Lymphocyte rich Hodgkin lymphoma. This type is rare and may be hard to distinguish from nodular lymphocyte predominant Hodgkin lymphoma. It usually begins in the lymph nodes of the neck, underarm, and groin, and sometimes involves the spleen and nodes in the chest.

Lymphocyte depleted Hodgkin lymphoma. This is a very rare, aggressive type that is uncommon in children. This type is usually more widespread, involving lymph nodes as well as the bones and bone marrow.

Age and Hodgkin lymphoma

There are three different forms of Hodgkin lymphoma:

A childhood form (in children 14 years or younger)

A young adult form (in people 15 to 34 years old)

An older adult form (in people 55 to 74 years old)

The childhood form of Hodgkin lymphoma is less common in the United States and is especially rare in children younger than five years. Hodgkin lymphoma is most commonly diagnosed in the young adult form and older adult form. In children younger than 5 years, Hodgkin lymphoma is more common in boys than in girls. Among adolescents, the rates of Hodgkin lymphoma are roughly equal among boys and girls.

Symptoms and Signs

Children with Hodgkin lymphoma may experience the following symptoms or signs. Sometimes, children with Hodgkin lymphoma do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom or sign on this list, please talk with your child’s doctor.

Painless swelling of lymph nodes in the neck, underarm, or groin that doesn’t go away in a few weeks

Coughing or problems breathing, which may mean that the lymph nodes in the chest are swollen and pressing on the windpipe

Unexplained fever (without other signs of infection) that doesn’t go away

Unexplained weight loss

Night sweats (usually drenching)

Itching

Fatigue

Your child’s doctor will ask you questions about the symptoms your child is experiencing to help find out the cause of the problem, called a diagnosis. This may include how long your child has been experiencing the symptom(s) and how often.

If cancer is diagnosed, relieving symptoms and side effects remains an important part of cancer care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your child’s health care team about symptoms your child experiences, including any new symptoms or a change in symptoms.

Risk Factors

A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors often influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do.

The cause of Hodgkin lymphoma is unknown, although infection with the Epstein-Barr virus may play a role in the development of the cancer in approximately 30% of children and teens. People with immune system problems also have a higher risk of developing Hodgkin lymphoma. This group includes:

Children born with the hereditary condition of ataxia telangiectasia, which causes immune system problems

Children with human immunodeficiency virus (HIV), the virus that causes acquired immune deficiency syndrome (AIDS)

Children who are taking drugs that suppress the immune system following an organ transplant.

Diagnosis

Doctors use many tests to diagnose cancer and find out if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most types of cancer, a biopsy is the only way to make a definitive diagnosis of cancer. Imaging tests may be used to find out whether the cancer has metastasized. Your child’s doctor may consider these factors when choosing a diagnostic test:

Age and medical condition

Type of cancer suspected

Severity of symptoms

Previous test results

The following tests may be used to diagnose Hodgkin lymphoma:

Physical examination/blood tests. Children tend to have larger lymph nodes than adults. Usually, a child has enlarged lymph nodes for several weeks or months before a doctor suspects Hodgkin lymphoma, which is uncommon in children. The doctor first looks for signs of a more common infection that may cause the lymph nodes to swell and may prescribe antibiotics.

If swelling in the lymph nodes does not go down after a course of antibiotics, the swelling may be caused by something other than an infection. In these instances, the doctor does a physical examination of all the lymph node areas, the liver, and the spleen, which may be enlarged in children with Hodgkin lymphoma.

Blood tests. may also be done to check blood counts and evaluate how the liver and kidneys are working. There is no specific blood test for Hodgkin lymphoma, but changes in blood counts (such as unexplained anemia or a low number of red blood cells) are sometimes more common in children with Hodgkin lymphoma.

Biopsy. If the lymph nodes don’t feel normal when the doctor examines them and don’t respond to antibiotics, the doctor will check tissue from the abnormal lymph node for cancer cells. Hodgkin lymphoma makes a distinctive kind of abnormal cell, called a Reed-Sternberg cell, that is easily identified under the microscope. The only way to diagnose Hodgkin lymphoma is to look at the tissue from an abnormal lymph node under the microscope. The process of removing the tissue is called a biopsy.

To perform a standard biopsy for Hodgkin lymphoma, a surgeon cuts through the skin and removes an entire lymph node or a piece of a mass of lymph nodes. In children, a lymph node biopsy is usually performed with general anesthesia or conscious sedation, in which the child is awake but the pain and discomfort are lessened with medication. 

Sometimes, a doctor may first try to obtain tissue from the lymph node by doing a fine needle aspiration biopsy. In this test, a thin needle is used to remove small amounts of fluid and tissue from the lymph node. This type of biopsy may not provide enough tissue to diagnose the disease, so it is recommended only when a standard, surgical biopsy may be too difficult or dangerous.

If a biopsy confirms the diagnosis of Hodgkin lymphoma, several tests and scans can help the doctor learn more about the disease, show how far the disease has spread , and indicate how well treatment is working. These scans and tests may include:

X-ray. An x-ray is a way to create a picture of the structures inside of the body using a small amount of radiation. For instance, a chest x-ray will show whether lymph nodes in the mediastinum are enlarged. Mediastinal tumors that take up one-third or more of the chest cavity are considered "bulky." They may cause coughing or breathing problems by narrowing the airway.

Computed tomography (CT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. A contrast medium (a special dye) is injected into a vein to provide better detail. The CT scan shows if lymph nodes in the chest or abdomen are enlarged, which may be a sign of cancer. Also, this test will show if the organs—such as the lungs, liver, or spleen—are involved.

Positron emission tomography (PET) scan. A PET scan is a way to create pictures of organs and tissues inside the body. A small amount of a radioactive substance is injected into a patient’s body. This substance is absorbed mainly by organs and tissues that use the most energy. Because cancer tends to use energy actively, it absorbs more of the radioactive substance. A scanner then detects this substance to produce images of the inside of the body.

PET scans are often used to add to the information gathered from a CT scan and physical examination. This test can also be used to evaluate how well treatment is working. Before treatment, areas of active Hodgkin lymphoma appear bright on the scan in most people. During and after treatment, these bright areas usually go away as the cancer cells are dying. This test can reassure families and doctors—without doing a biopsy—that scar tissue still present on a CT scan after treatment does not contain active cancer cells.

Bone marrow biopsy. Hodgkin lymphoma rarely spreads to the bone marrow in children with localized Hodgkin lymphoma located only in the lymphatic system. A bone marrow biopsy is recommended for children with signs of more widespread disease involving lymph glands above and below the diaphragm. It is also recommended for children with other signs of Hodgkin lymphoma that has spread outside the lymph node system to the lungs, liver, or bones, who are more likely to have lymphoma in the bone marrow.

For a bone marrow biopsy, the child’s skin is numbed with a local anesthetic, or—more commonly—conscious sedation is given while a needle is inserted into the bone in the hip until it reaches the spongy part of the bone at the center, the bone marrow. A small amount of marrow is removed and examined under a microscope.

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium (a special dye) may be injected into a patient’s vein to create a clearer picture. This test may be used instead of or in addition to a CT scan at diagnosis or during follow-up care to check for lymphoma in the abdomen, bones, or lymph nodes in the chest. 

Stages and Grades

Staging is a way of describing where the cancer is located, if or where it has spread, and whether it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer's stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis (chance of recovery). There are different stage descriptions for different types of cancer.

After the diagnostic tests are completed, the doctor will assign a stage, which is needed to plan treatment. The four stages of Hodgkin lymphoma (I to IV; one to four) are described below.

In addition, each person’s disease is put into one of two categories, A or B, based on whether the person has symptoms of unexplained fever, drenching night sweats, or weight loss in the six months before diagnosis. A means the patient does not have these symptoms, while B means that the patient has at least one of these symptoms.

Stage I: Cancer is in only one area of the lymph nodes or in one area or organ outside of the lymph nodes.

Stage II: Cancer is in two or more lymph node areas on the same side of the diaphragm, or cancer is in one lymph node area and in one area or organ next to the lymph nodes.

Stage III: Cancer is in lymph node areas above and below the diaphragm. The cancer may have spread to an area or organ near these lymph nodes and, possibly, to the spleen.

Stage IV: Cancer has spread outside of the lymph node system to the lungs, liver, bones, bone marrow, or other organs.

Recurrent: Recurrent cancer is cancer that comes back after treatment. If there is a recurrence, the cancer may need to be staged again (re-staging) using the system above.

Risk grouping

A treatment regimen (schedule) may be selected based on the disease’s classification as low, intermediate, or high risk. This classification is based on several factors, including the cancer’s stage, whether the tumor is bulky, and whether the patient is experiencing specific symptoms (defined as A or B; see above).

Children with low risk Hodgkin lymphoma usually have stage IA or stage IIA disease without bulky tumors.

In high risk Hodgkin lymphoma, the cancer is in a later stage or is causing B symptoms like fever, drenching night sweats or weight loss. This could include stages IIIB and IVB.

Other patients are usually considered intermediate risk.

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