Hodgkin Lymphoma (Disease) in Children – Just Diagnosed

Diagnosing Hodgkin Lymphoma

A number of tests are performed to evaluate a child who is suspected of having Hodgkin lymphoma. Evaluation involves a combination of physical examination, laboratory studies and imaging studies. Diagnosis usually requires:

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Determining the Extent of Hodgkin Lymphoma

Hodgkin lymphoma has a standardized staging system (stages I-IV). 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.

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 IIIES: 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 diffusely distributed through sites usually outside of the lymph nodes, with or without lymph node involvement. When cancer is found diffusely in tissues such as the lungs, liver, bone, or bone marrow it is classified as Stage IV.

In each stage of Hodgkin lymphoma, the disease receives a further classification 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.

For each stage, a sub-classification of X is assigned if “bulky disease” is present. Bulky disease is defined by an abdominal node or mass equal to or greater than 10 cm in size, or a thoracic mass equal to or greater than one-third of the transverse diameter of the thorax.

At the time of diagnosis, approximately 60% of children have stage I or II disease, 30% have stage III disease, and 10% have stage IV disease.

Causes of Hodgkin Lymphoma

Researchers are aware of a few risk factors for Hodgkin lymphoma. They include Epstein Barr Virus (EBV), the virus responsible for mononucleosis. In about 20-50% of Hodgkin disease patients, the tumor shows evidence of EBV. However, it is extremely rare that children who have had EBV will develop Hodgkin lymphoma.

Siblings, parents, and children of young adults with Hodgkin lymphoma have a slightly 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.

Hodgkin lymphoma is found more frequently in children of higher socioeconomic status and in children from smaller families.

Hodgkin lymphoma is more common in children with weakened immune systems. Children who have received organ transplants and people with HIV infection are at increased risk of developing Hodgkin lymphoma.

Prognosis of Hodgkin Lymphoma

The prognosis for children and young adults with Hodgkin lymphoma depends on a number of factors, including treatment and the stage of the disease at diagnosis. Almost all children with stage I or II disease can be cured. About 1 in 10 children with early stage disease may have a disease course that requires additional therapy.

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
  • Low red blood cell count
  • Male gender
  • High white blood cell count

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

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