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Hodgkin disease (also known as Hodgkin lymphoma) is a cancer of the lymphoid system. The lymphoid system is made up of various tissues and organs, including the lymph nodes, tonsils, bone marrow, spleen and thymus. These organs produce, store, or carry white blood cells to fight infection and disease.

Approximately 900 children and adolescents each year are diagnosed with Hodgkin disease in the United States. It has been reported in infants and very young children, but is considered rare before the age of five. The number of cases increases significantly in the second decade of life, making it most common in teenagers.

Most patients who have Hodgkin disease experience swollen lymph nodes (small tissues located throughout the body that store white blood cells to help filter out germs), called lymphadenopathy. People usually cannot feel lymph nodes unless they become swollen while fighting infection. In Hodgkin disease, the lymph nodes are usually larger than those that occur with common infections, and they do no shrink when treated with standard medications like antibiotics. The affected lymph nodes are usually found in the neck or above the collarbone, and less commonly under the arms or in the groin. The lymph nodes are usually painless, firm, rubbery, and movable in the surrounding tissue.

Poor appetite, weight loss, night sweats, fever, a sense of ill-feeling, and fatigue may also be experienced by children with Hodgkin disease. They are "systemic" symptoms with a more generalized affect on the entire body. Itching (pruritus) is another symptom in patients with Hodgkin disease. Itching can be mild or severe, and tends to occur more often in patients with advanced disease.

Diagnosing Hodgkin Disease

Evaluation begins with a complete medical history and physical examination. The physician will examine your child's lymph nodes and abdomen thoroughly. The presence of small, soft lymph nodes are less concerning than nodes that have been increasing in size or are significantly enlarged. An enlarged spleen or liver may indicate generalized disease. While a chest X-ray is usually performed prior to any biopsy, a lymph node (or rarely other site) biopsy is needed to diagnose the disease.

A number of tests are performed to evaluate a child who is suspected of having Hodgkin disease. Evaluation involves a combination of physical examination, laboratory studies and imaging studies. Your child's pediatrician or your family doctor may order some initial tests before referring you to a specialist. Some of the tests may need to be repeated to better establish the diagnosis.

• Laboratory studies (do not establish stage of disease but are important)
  • A  complete blood count (CBC)
  • Erythrocyte sedimentation rate (ESR)—a blood test that detects inflammation in the body.
  • Kidney and liver function tests
• Imaging Studies (not all tests are needed for every patient)
  • Chest X-ray
  • Computer tomography (CT) scan
  • Magnetic resonance imaging (MRI)
  • Bone scan
  • PET scan
  • Bone marrow biopsy

Determining the Extent of Hodgkin Disease

A standardized staging system (stages I-IV) developed for Hodgkin disease is called the Ann Arbor staging system. The stages are defined by the locations of lymph nodes in the body that are infiltrated by the cancer. The higher the stage number, the more the disease has spread throughout the lymphoid system and into other parts of the body.

Ann Arbor Staging Classification for Hodgkin Disease

Stage I: Cancer is found in one location.

• Stage I: Cancer is found in a single lymph node region (most commonly in the neck).
• Stage IE: Cancer is found in one organ or site outside the lymph nodes.

Stage II: Cancer is found in more than one location, but the cancer sites are located either entirely above or below the diaphragm (the muscle located below the lungs).

• Stage II: Cancer is limited to two or more lymph node regions on the same side of the diaphragm.
• Stage IIE: Cancer is found in one or more lymph node regions and a nearby organ or site on the same side of the diaphragm.

Stage III: Cancer is found both above and below the diaphragm.

• Stage III: Cancer is limited to the lymph nodes, but affected nodes are on both sides of the diaphragm.
• Stage IIIE: Cancer is found in lymph nodes on both sides of the diaphragm, as well as in a nearby organ or site.
• Stage IIIS: Cancer is found in lymph nodes on both sides of the diaphragm, as well as in the spleen.
• Stage IIIE+S: Cancer is found in lymph nodes on both sides of the diaphragm, as well as in the spleen and a nearby organ or site.

Stage IV: Cancer is located in sites usually outside of the lymph nodes. When cancer is found in the lungs, liver, bone or bone marrow it is classified as Stage IV.

Each stage is further classified as either A or B.

• A refers to disease without any of the 3 “B” symptoms
• B symptoms include:
• Unexplained recurrent fever exceeding 100.4˚F / 38.0˚C,
• Drenching night sweats, and
• Unintentional weight loss of at least 10% of body weight over 6 months.

At the time of diagnosis, approximately

• 60% of children have stage I or II disease
• 30% have stage III disease
• 10% have stage IV disease

Causes of Hodgkin Disease

The causes of Hodgkin disease are unknown. Studies that examine the rates of disease within populations, called epidemiologic studies, suggest some possible factors that may be at work. However, there is no evidence that any one particular factor is responsible for this disease. According to the current state of medical knowledge, the following things most likely DO NOT increase a child’s risk of Hodgkin lymphoma:

• Contact with someone who has Hodgkin lymphoma
• Ionizing radiation
• Chemical exposures

Only a few risk factors for Hodgkin disease are known for sure.

Epstein Barr Virus (EBV):EBV, the virus responsible for mononucleosis, is seen in the tumors of 20-50% of Hodgkin disease patients. Researchers believe that EBV is part of the cause of Hodgkin disease in these patients. However, of people who were infected with EBV in childhood or adolescence, only a few get Hodgkin lymphoma. When EBV is not found in the Hodgkin disease tumor, the virus is not part of the cause. Although researchers know that EBV infection is related to some cases of Hodgkin disease, they do not know why a few people who have been infected with EBV develop Hodgkin disease while the great majority do not.

Family history of Hodgkin disease: Siblings, parents, and children of young adults with Hodgkin lymphoma are at increased risk of having the cancer. Studies have reported an increased risk of disease especially among same-sex siblings of patients with Hodgkin disease. Although the risk of having Hodgkin disease is two-to five-times greater for siblings, the likelihood that a sibling will get the cancer remains extremely low. The clustering of cases of Hodgkin disease within families and in certain ethnic groups may suggest a genetic predisposition to the disease.

Economic status:Compared to residents of poor countries, residents of developed countries like the U.S. are less likely to develop Hodgkin disease when under 10 years old, but more likely to develop the cancer as a young adult.

Social contacts:Having fewer siblings and playmates as a child appears to increase the risk of developing Hodgkin disease as a young adult.

Immune system deficiencies:Hodgkin lymphoma is more common in people whose immune system is weakened. Immune deficiency can be caused by genetic conditions or can be acquired. Therefore, people who have received organ transplants and people with HIV infection are at increased risk.

Determining Treatment and Likelihood of Survival

The type of treatment and likelihood of long-term survival depend on many factors. Almost all children with stage I or II disease can be cured; although about 1 in 10 children with early stage disease may develop new disease and need additional therapy. The recurrence of the disease decreases the chance of long-term survival.

The presence of the following factors can decrease the chance of long-term survival:

• Advanced stage of disease (stage III or stage IV). About 8 in 10 children with stage III and IV disease can be cured.
• B symptoms at the time of diagnosis. About one-third of the children staged for Hodgkin disease have the B classification. This percentage increases in advanced disease.
• A large, cancerous lymph node or group of nodes, also referred to as “tumor bulk."
• Histology – the way the tumor looks under the microscope.

Children with these findings may need more intense therapy and radiation therapy to have the best chance of a cure.

Last updated September, 2011

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