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Non-Hodgkin lymphoma

Overview

Lymphomas are tumours which form in the lymphatic system, which is made up of the spleen, bone marrow, thymus, tonsils, lymph nodes and lymph vessels.

Non-Hodgkin lymphomas are the most common lymphoma in children. They can be fast or slow growing, and formed by either B-cells or T-cells. Lymphoma cells are often further defined by their size (large or small), their shape (seen under a microscope), and their growth pattern (scattered or clustered).

The main types of non-Hodgkin lymphomas are:

• Large B-cell lymphoma: the most common type of non-Hodgkin lymphoma, usually fast-growing, and characterised by rapidly growing tumors in the spleen, lymph nodes, liver, bone marrow, or other organs.
• Anaplastic large cell lymphoma: a fast-growing lymphoma that is usually of the T-cell type.
• Lymphoblastic lymphoma: a fast-growing lymphoma where immature white blood cells (called lymphoblasts) spread through the lymph nodes and the thymus gland. This can occur to both B or T-cells.
• B-cell non-hodgkin lymphoma, or Burkitt lymphoma.

The survival rate for non-Hodgkin lymphoma in children is high, with approximately 85% of children in Australia surviving five years or more after treatment.

Risk Factors

As with most childhood cancers, the causes of non-Hodgkin lymphoma are not yet fully understood. However, there are some known risk factors for this disease, including certain inherited immune deficiencies, previous cancer treatment or organ transplant, and HIV infection.

Cancer researchers are making steady progress in understanding the gene changes that cause lymphoma cells to develop, and why these cells fail to develop and mature normally. This information is being applied to develop new treatments for lymphoma, as well as improved tests for detecting and monitoring this disease.

Symptoms

Non-Hodgkin lymphoma may cause different signs and symptoms, depending on where the cancer originates or spreads.

Non-Hodgkin lymphoma often starts in the abdomen, the lymph nodes on the sides of the neck, in the underarm area, above the collar bone, or in the groin area. It can also develop in the brain or spinal cord, bone marrow, or skin.

Along with signs arising in the part of the body where it develops, such as a lump, on-Hodgkin lymphoma can also cause general symptoms such as:

• fever and chills
• difficulty breathing
• sweating (particularly night sweats)
• weight loss.

Diagnosis

Non-Hodgkin lymphoma is diagnosed by:

• physical examination (e.g. looking for enlarged lymph nodes)
• biopsy (removing cells from the lump or lymph node for microscopic examination).

In a child with suspected lymphoma, imaging tests (e.g. x-ray, ultrasound, magnetic resonance imaging or MRI) may also be done to look more closely at an abnormal area, and to learn how far the lymphoma has spread.

Assessing the extent of spread of non-Hodgkin lymphoma is called staging. Stages range from Stage I (the least advanced) to Stage IV (the most advanced). The stage will affect both a child’s treatment and prognosis.

Treatment

Treatment for non-Hodgkin lymphoma may be required for up to two years. Chemotherapy is the main treatment used for all types of non-Hodgkin lymphoma, although each type of non-Hodgkin lymphoma may be treated slightly differently.

Lymphoblastic lymphoma is often treated more intensively than other types of lymphoma, while treatment for Burkitt lymphoma may include a monoclonal drug and/or stem cell therapy as well as chemotherapy. Large cell lymphomas (anaplastic large cell lymphoma, and large B-cell lymphoma), which can grow almost anywhere in the body, may be treated with surgery, chemotherapy, radiotherapy, and/or stem cell transplant.

Follow Up

Children treated for non-Hodgkin lymphoma should have regular follow-up visits with their medical team for several years. These check-ups may include physical examination, lab tests, and imaging tests, and are focused on looking for signs of lymphoma, as well as for any long-term side effects of treatment (late effects).

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