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Hodgkin Disease (Hodgkin lymphoma)

Overview

Lymphomas are tumours of the lymphatic system – the spleen, bone marrow, thymus, lymph nodes and lymph vessels. Since lymph tissue is found all around the body, Hodgkin lymphoma can have a number of potential start points and can spread to almost any tissue or organ.

Hodgkin lymphoma (also known as Hodgkins lymphoma or Hodgkins Disease), most commonly affects children, and particularly, teenagers between 14-19 years old.

There are two main types of Hodgkin lymphoma: classical Hodgkin lymphoma, and nodular lymphocyte predominant Hodgkin disease. Classical Hodgkin lymphoma accounts for 95% of cases in Australia, and has 4 subtypes which are classified based on how they look under a microscope.

Survival for early stage Hodgkin lymphoma in Australia is more than 90%, and for late stage HD is more than 85%.

Risk Factors

The risk of Hodgkin lymphoma is thought to be increased if a child contracts a disease that weakens their immune system.

Risk factors for Hodgkin lymphoma include:

• infection with the Epstein – Barr virus
(the cause of infectious mononucleosis, also known as glandular fever)
• infection with HIV (human immunodeficiency virus)
• certain inherited diseases of the immune system
• having a parent or sibling with a previous diagnosis of Hodgkin lymphoma.

Symptoms

Classical Hodgkin lymphoma usually first appears as a painless swelling of one gland, or a group of lymph glands – often in the neck, chest, underarm or groin. (It’s important to remember that glands can also become swollen with common infections, such as those causing a sore throat or a cold.)
If glands in the chest are affected, a cough or breathlessness can result due to pressure on the airways.

Some children with HD also have other symptoms, called B symptoms. These can include:

• fever
• drenching night sweats
• weight loss (10% of body weight or more over 6 months)
• loss of appetite.

Diagnosis

Diagnosis of Hodgkin lymphoma is done by physical examination (e.g. feeling swollen lymph nodes) and biopsy (removing cells from the lump or lymph node for microscopic examination).

Patients may also undergo imaging tests such as x-rays, ultrasounds, CT scans, PET scans and blood tests to determine how far the lymphoma has spread throughout the body.

The process used to determine how far cancer has spread is called staging. Stages for Hodgkin lymphoma are a combination of stages 1-4, and stages A, B, E and S.

A and B refer to the presence or absence of B symptoms, while E indicates whether cancer is found in an organ or tissue that is not part of the lymph system. A stage that includes the letter ‘S’ will indicate that the cancer has been found in the spleen.

Treatment

Treatment for Hodgkin lymphoma is based on the type and location of the disease, and its stage, as well as factors such as the age and general health of the child. In some cases, treatment for teenagers and young adults may more closely resemble an adult treatment regime. If the Hodgkin Lymphoma is in its early stages and is considered relatively low-risk, it will be treated with a selection of anti-cancer chemotherapy drugs, and occasionally paired with radiation therapy.

As the severity of the cancer increases, so will the dosage of recommended chemotherapy and the treatment period. Sometimes a patient’s stem cells will be extracted before chemotherapy, and then stored so they can be used afterwards in a stem cell transplant.

Treatments for HD are a topic of ongoing research. In particular, researchers are trying to find a way of clearly identifying which patients can be treated with a less aggressive form of treatment, so that not all children need be placed at risk of serious side-effects. Researchers are also trying to develop less-toxic treatments, and several new drug combinations are being studied.

Follow Up

As with other types of cancer, children treated for Hodgkin lymphoma need to have regular follow-up visits to hospital for several years. There are a number of potential side effects that can appear months or years after a child’s cancer treatment regime has ended (late effects), and can include issues with physical development (fertility, bone and muscle growth, heart or lung conditions) as well as changes in mood, learning or memory.

Secondary cancers are also a risk.

Check-ups after cancer treatment may include physical examination, and imaging tests, and are aimed at finding any signs of cancer, as well as any late effects, so that these can be addressed effectively.

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