Hodgkin disease, or Hodgkin lymphoma, is a type of cancer that affects the lymphatic system. This highly sophisticated filtering system helps the body's immune system remove bacteria, viruses, and other unwanted substances.
The lymphatic system includes the lymph nodes (glands), thymus, spleen, tonsils, adenoids, and bone marrow, as well as the channels (called lymphatics or lymph vessels) that connect them. When lymphoma occurs, cancer cells develop in a person's lymphatic system and start to grow uncontrollably.
The different types of lymphomas are grouped into two broad categories: Hodgkin disease and non-Hodgkin lymphoma. Lymphomas that involve a type of cell known as a Reed-Sternberg cell are classified as Hodgkin disease. The different subtypes of Hodgkin disease are classified based on how the cancerous tissue looks under a microscope.
Lymphomas without Reed-Sternberg cells are usually classified as non-Hodgkin lymphomas.
Hodgkin disease tends to occur more often in teens and in people with severe immune deficiencies from things such as inherited immune defects, human immunodeficiency virus (HIV) infection, or treatment with immunosuppressive drugs after organ transplants.
Although no lifestyle factors have been definitely linked to childhood lymphomas, kids who have received either radiation treatments or chemotherapy for other types of cancer seem to have a higher risk of developing lymphoma later in life.
Usually, the causes of lymphoma are unknown. So, neither parents nor kids have control over the factors that cause the disease. Most lymphomas come from noninherited mutations (errors) in the genes of growing blood cells. Regular pediatric checkups can sometimes spot early symptoms of lymphoma.
Signs and Symptoms
The most common first symptom of Hodgkin disease is a painless enlargement of the lymph nodes (a condition known as swollen glands) located in the neck, above the collarbone, in the underarm area, or in the groin.
If cancer involves the lymph nodes in the chest, pressure may trigger an unexplained cough, shortness of breath, or problems in blood flow to and from the heart.
About a third of patients have other nonspecific symptoms, including fatigue, poor appetite, itching, or hives. Unexplained fever, night sweats, and weight loss are also common.
One of the things doctors might look for if they suspect lymphoma is enlargement of the lymph nodes. They may first try to treat swollen lymph nodes with antibiotics, because infections are the most common cause of swollen lymph nodes. But if the lymph nodes remain swollen, a child may be referred to an oncologist (cancer doctor), who may recommend a biopsy (tissue sample) of the lymph nodes.
During a biopsy, a tiny bit of tissue is removed from the body and sent out to a laboratory for analysis. If Hodgkin disease is suspected, either of the following biopsies may be performed:
- Core biopsy: the doctor uses a hollow needle to remove a small amount of tissue from the lymph node.
- Excisional or incisional biopsy: the doctor opens the skin to remove the entire enlarged lymph node (excisional) or only part of it (incisional).
Depending on the type of biopsy ordered, the doctor may use local anesthesia (where only a part of the body is numbed) or general anesthesia (which puts a patient to sleep) to ensure that the child doesn't feel any pain.
If the doctor suspects Hodgkin disease, more tests will be performed to determine if the cancer has spread. These can include:
- blood tests
- a chest X-ray
- a computed tomography (CT or CAT) scan, a type of X-ray that rotates around the patient and creates a picture of the inside of the body from different angles
- a bone marrow biopsy to check for cancer in the bone marrow
- a gallium scan, which uses the injection of a chemical called gallium to detect tumors and inflammation
- a positron emission tomography (PET) scan, which can tell the difference between normal and abnormal cells based on their metabolic activity
In rare cases, doctors may perform surgery to further determine the stage of the cancer. One such surgical test is a laparotomy, in which a child is put to sleep and a surgeon operates to examine the internal organs. But such surgical procedures are becoming rare given the wide variety of imaging technologies available today.
Treatment for Hodgkin disease is very effective for most kids. The choice of treatment is largely determined by staging, a way to categorize or classify patients according to how extensive the disease is at the time of diagnosis.
There are four stages of lymphoma, ranging from Stage I (cancer involving only one area of lymph nodes or only one organ outside the lymph nodes) to Stage IV (cancer has spread, or metastasized, to one or more tissues or organs outside the lymphatic system). The stage at diagnosis can guide medical professionals in the decision of therapy and helps doctors predict how someone with lymphoma will do in the long term.
Treatment may involve radiation (the use of high-energy rays to shrink tumors and keep cancer cells from growing), chemotherapy (the use of highly potent medical drugs to kill cancer cells), or both, depending on the type and stage of the cancer as well as the age and overall health of the child.
For children who whose cancer comes back after treatment or who do not respond well to treatment, doctors may perform bone marrow or stem cell transplants to replace cells damaged by chemotherapy or radiation. These transplants involve taking the cells from bone marrow or blood (either taken from the patient or donated by another person) and transplanting them to the patient after chemotherapy treatment.
Short-Term and Long-Term Side Effects
Treatment for Hodgkin disease is powerful. It destroys good cells along with bad, which can create certain side effects.
Intensive lymphoma chemotherapy affects the bone marrow, causing anemia and bleeding problems, and increasing the risk for serious infections. Chemotherapy treatments have side effects — some short-term (such as hair loss, increased infection risk, and nausea and vomiting) and some long-term (such as heart and kidney damage, reproductive problems, or the development of another cancer later in life) — that parents should discuss with the doctor.
Side effects of radiation include fatigue, loss of appetite, and skin reactions.
The majority of kids with Hodgkin disease are cured, meaning they will have long-term cancer-free survival.
Most children go into remission (where there is no longer evidence of cancer cells in the body) following initial chemotherapy. A long-term cure is achieved in almost all Stage I or Stage II patients, and even in the majority of Stage III and Stage IV patients. However, those with higher-stage disease are at risk for more long-term side effects as a result of more intensive treatment.
Reviewed by: Robin E. Miller, MD
Date reviewed: January 2009