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Hodgkin Lymphoma

What is Hodgkin Lymphoma?

Hodgkin lymphoma is a cancer of the lymphatic system. The lymphatic system is part of the immune system.

It consists of organs and tissues, including:

  • Lymph nodes
  • Lymphatic vessels
  • Tonsils
  • Thymus
  • Bone marrow
  • Spleen

These tissues produce, store, and carry white blood cells called lymphocytes. They fight infection and disease.

Hodgkin lymphoma is a cancer of the lymphatic system, which is part of the immune system. The lymphatic system is made up of organs and tissues, including the lymph nodes, lymphatic vessels, tonsils, bone marrow, spleen and thymus. These tissues produce, store, or carry white blood cells called lymphocytes that fight infection and disease.

There are two types of lymphoma: Hodgkin and non-Hodgkin.

The main difference is the type of lymphocyte involved. The lymphoma is Hodgkin lymphoma if testing on the biopsy tissue show certain tumor markers along with larger atypical cells called the Hodgkin Reed-Sternberg cell. Reed-Sternberg cells are distinctive because they have two nuclei. Some say these cells have the appearance of “owl eyes.”

This illustration shows a boy with organs of the lymphatic system labeled: Cervical nodes, lymph vessels, axillary nodes, inguinal nodes, spleen, thymus, and tonsils.

The lymphatic system is made up of organs and tissues, including the lymph nodes, lymphatic vessels, tonsils, bone marrow, spleen and thymus.

Reed-Sternberg cells, which have the appearance of owl eyes, shown in a teal circle.

Reed-Sternberg cells are distinctive because they have two nuclei, which some say have the appearance of “owl eyes.” The presence of Reed-Sternberg cell classifies the lymphoma as Hodgkin lymphoma.

Subtypes of Hodgkin Lymphoma

Within Hodgkin lymphoma, there are two major subtypes: classical and nodular lymphocyte predominant.

  • Classical Hodgkin lymphoma is the most common. There are 4 kinds of classical Hodgkin lymphoma and all are positive for the tumor marker CD30.
    • Lymphocyte-rich
    • Nodular sclerosis
    • Mixed cellularity
    • Lymphocyte-depleted
  • Nodular lymphocyte predominant Hodgkin lymphoma. This subtype is positive for the tumor marker CD20 and is negative for CD30.

Knowing the specific type of Hodgkin lymphoma is very important to help doctors prescribe the best type of treatment. All kinds of classical Hodgkin lymphomas are treated the same. Nodular lymphocyte-predominant Hodgkin lymphoma has different markers. It is more slow-growing. It allows for a different treatment approach.

How common is Hodgkin lymphoma?

Some 6,000-7,000 new cases of Hodgkin lymphoma are diagnosed each year in the United States.

What are the causes/ risk factors for childhood Hodgkin lymphoma?

Causes and risk factors include:

  • Age: Hodgkin lymphoma in childhood most often affects teens ages 15-19. It also is more common in young adults and adults older than 50.
  • Viral infections: Epstein-Barr virus (EBV), the virus that causes mononucleosis, has been linked to Hodgkin lymphoma. Many people get EBV. But very few develop Hodgkin lymphoma.
  • Weakened immune system: A person with a weakened immune system can be at higher risk. Immune problems may be caused by an inherited disease, certain medications, or human immunodeficiency virus (HIV) infection.
  • Family history: Having a brother, sister, or parent with Hodgkin lymphoma slightly elevates the risk of developing Hodgkin lymphoma. But it does not make it harder to treat.

Signs and Symptoms of Hodgkin Lymphoma

The most common symptom is painless, swollen lymph nodes in the neck, chest, armpit, or groin area.

Other common symptoms include:

  • Weight loss of more than 10 percent of total body weight over 6 months for no other reason
  • Drenching night sweats (so severe that bed sheets or pajamas may need to be changed)
  • Fevers more than 100.4 degrees Fahrenheit (38 degrees Celsius) for 3 or more consecutive days that have no known cause
  • Unexplained cough or shortness of breath

Diagnosis of Hodgkin Lymphoma

  • Physical exam and medical history
  • Blood tests
  • X-ray and other imaging tests
  • Biopsy
  • Bone marrow tests
  • Tests to see if the cancer has spread

A biopsy of lymph node tissue is required to make a diagnosis of Hodgkin lymphoma.

Biopsy

A surgeon will perform a biopsy to remove tissue from an enlarged lymph node. Pathologists examine the tissue under a microscope and provide a diagnosis.

The type of biopsy depends on the location of the suspected cancer:

  • Excisional biopsy: The removal of an entire lymph node or lump of tissue. This type of biopsy is preferred. The pathologist can see the architecture of the node to help make the diagnosis.
  • Incisional biopsy: The removal of part of a lump, lymph node, or tissue sample.
  • Core biopsy: The removal of small pieces of tissue or part of a lymph node using a wide needle.
  • Fine-needle aspiration biopsy: The removal of tissue or part of a lymph node using a thin needle.

If Hodgkin lymphoma involves lymph nodes deep in the chest, a biopsy may involve a mediastinoscope. It is a thin, tube-like instrument used to examine and remove tissue and lymph nodes in the area between the lungs. Doctors will use this method if there are no other lymph nodes that are easier to sample.

Analysis of biopsy tissue

The pathologist will examine the tissue to look for cancer cells. Hodgkin Reed-Sternberg cells indicate classical Hodgkin lymphoma.

If abnormal cells are present, the pathologist will perform more testing on the tissue sample to look for specific markers present on the atypical cancer cells. This process can take a few days.

Doctors will conduct more tests to determine the stage of the disease. The stage indicates where the cancer is located in the body.

The meaning of the stage in Hodgkin lymphoma is a little different because lymph nodes throughout the body are connected. Cancer may appear in several or many places, but it does not make it harder to treat or more risky like in other types of cancer. Staging in Hodgkin lymphoma depends on:

  • The number of affected lymph node regions
  • The patient’s signs and symptoms
  • If the tumor is bulky
  • If lymphoma has spread outside the lymphatic system
Stage Where Cancer is Found
Stage 1 In 1 or more lymph nodes in one lymph node group
Stage 2 In 2 or more lymph node groups only on one side of the diaphragm. Either above OR below.
Stage 3 In lymph node groups above AND below the diaphragm
Stage 4 In areas of the body that are not part of the lymph node system, such as the liver, lungs, or bone marrow. The cancer has spread to these areas from more distant places in the body.

A or B designations are also used in addition to the stage for every patient.

A means you have no “B” symptoms.

B is added if a patient has the presence of at least one of the following symptoms:

  • Drenching night sweats
  • Unexplained fevers with temperature more than 38°C or 100.4°F for 3 consecutive days
  • More than 10% loss of total body weight during the past 6 months for no other reason

E is used when the cancer is found outside the lymph system in one organ or area.

Imaging tests for staging at the time of initial diagnosis may include:

Bone marrow aspiration and biopsy

A bone marrow aspiration and biopsy may be performed to see if cancer is located in the bone marrow. Sometimes imaging alone can provide this information and the procedure is not needed.

Risk groups

Risk groups are determined at diagnosis and are used to plan treatment. Doctors determine risk groups based on the signs, symptoms, and stage of the cancer. All risk groups have the same outcomes if they receive the appropriate treatment based on risk grouping. This is why it is so important to accurately identify the risk group through correct staging.

Risk groups:

  • Low
  • Intermediate
  • High

Treatment of Hodgkin Lymphoma

Prognosis of Hodgkin Lymphoma

The survival rate for Hodgkin lymphoma in the U.S. is more than 95 percent.

Late Effects of Hodgkin Lymphoma Treatment

Cancer treatments may have long-term and late effects.

Long-term effects start during treatment and continue after treatment ends.

Late effects don’t start until later in life.

Long-term and late effects depend on what medicines were used, amount and location of radiation treatment, and age of the patient.

Focus of Current Hodgkin Lymphoma Research

Research continues to develop treatments that reduce the long-term and late effects in cancer survivors.

Research also focuses on developing more effective treatments for rare cases when children’s cancer doesn’t respond to the original therapy or returns after treatment.


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Reviewed: September 2019