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Non-Hodgkin Lymphoma

What is non-Hodgkin lymphoma?

Non-Hodgkin lymphoma is the group name for all types of lymphomas except Hodgkin lymphoma. Lymphomas are cancers that begin in the body’s lymphatic system, which is part of the body’s immune system.

The lymphatic system moves infection-fighting white blood cells called lymphocytes throughout the body. As a result, lymphoma can start in many places, including the: 

  • lymph nodes
  • thymus gland
  • spleen
  • abdomen
  • liver
  • bone marrow
  • skin
  • bones

Most non-Hodgkin lymphomas of childhood are different from adult lymphomas. Childhood lymphomas are typically more aggressive, but they are highly treatable.

How common is non-Hodgkin lymphoma in children and teens?

In the United States, about 800 new cases of non-Hodgkin lymphoma in children and teens are diagnosed each year.

This illustration shows a boy with organs of the lymphatic system labeled: Cervical nodes, lymph vessels, axillary nodes, inguinal nodes, spleen, thymus, and tonsils. Non-Hodgkin lymphoma is the group name for all types of lymphomas except Hodgkin lymphoma.

As part of the immune system, the lymphatic system moves infection-fighting white blood cells through the body.

There are several different types of non-Hodgkin lymphoma. Types of childhood non-Hodgkin lymphoma include:

Signs and symptoms of non-Hodgkin lymphoma

Signs and symptoms vary depending on where the disease starts in the body:

  • Cough and respiratory problems
  • Stomach pain
  • Nausea
  • Vomiting
  • Paleness of skin
  • Bone pain
  • Sores on the skin

Diagnosis of non-Hodgkin lymphoma

Doctors may begin to suspect cancer based on the child’s symptoms and from considering the results of a physical examination, medical history and blood tests. Sometimes doctors will order certain diagnostic imaging tests.

These tests may be completed at the local doctor’s office, hospital, or at a cancer center.

Biopsy

Doctors order a biopsy when cancer is suspected. A biopsy is required for diagnosis and will determine exactly what type of lymphoma. During a biopsy, tissue from the suspected tumor is removed and then examined by a pathologist under a microscope.

One of the following types of biopsies may be performed, depending of where the suspected cancer is located:

  • Excisional biopsy: The removal of a lymph node or lump of tissue.
  • Incisional biopsy: The removal of part of a lump, lymph node, or tissue sample.
  • Core biopsy: The removal 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. (NCI)

If lymphoma cells are present, additional tests will be performed on the tissue biopsy to diagnose the specific type of lymphoma. These tests include

  • Immunophenotyping
  • Cytogenetic analysis

Staging non-Hodgkin lymphoma

If a biopsy confirms cancer, doctors will perform more tests to determine the stage of the disease. The stage indicates whether the cancer has spread to other parts of the body and, if so, how much and how far it has spread.

The care team plans treatment based on the stage of the cancer. It is very important that lymphoma is staged as quickly as possible because these tumors grow rapidly. The higher the stage of the cancer, the more aggressively it needs to be treated.

  • Stage 1 and Stage 2 lymphomas are considered limited disease
  • Stage 3 and Stage 4 lymphomas and considered advanced.
Stage Where Cancer is Found
Stage 1 In one group of lymph nodes
In one area outside the lymph nodes
No cancer is found in the abdomen or area between the lungs
Stage 2 In one area outside the lymph nodes and in nearby lymph nodes
In two or more areas either above or below the diaphragm, and may have spread to nearby lymph nodes
To have started in the stomach or intestines and can be completely removed by surgery. Cancer may have spread to certain nearby lymph nodes.
Stage 3 In at least one area above the diaphragm and in at least one area below the diaphragm
To have started in the chest
To have started in the abdomen and spread throughout the abdomen
In the area around the spine
Stage 4 In the bone marrow
In the brain
Cerebrospinal fluid
Other parts of the body.
This illustration shows the areas of the body affected by disease in each stage of Non-Hodgkin Lymphoma.

The stage of lymphoma indicates whether the cancer has spread to other parts of the body and, if so, how much and how far it has spread.

Diagnostic imaging tests

Chest X-ray with evidence of non-Hodgkin lymphoma in pediatric patient.

Chest X-ray with evidence of non-Hodgkin lymphoma in pediatric patient.

Chest X-ray of pediatric non-Hodgkin lymphoma patient shows evidence of disease

Chest X-ray of pediatric non-Hodgkin lymphoma patient shows evidence of disease.

Lateral X-ray of pediatric non-Hodgkin lymphoma with evidence of disease

Lateral X-ray of pediatric non-Hodgkin lymphoma with evidence of disease.

Additional tests

Tests that may be performed to help stage childhood non-Hodgkin lymphoma include:

A screening test for HIV (human immunodeficiency virus) is also recommended because lymphomas sometimes develop in patients with this virus. If the test is positive, the patient may require medications to treat HIV.

Treatment of Non-Hodgkin Lymphoma

When determining the best treatment, doctors consider many factors. These include:

  • Subtype of the lymphoma
  • Stage of the lymphoma
  • How likely it is to respond to different treatments
  • Treatment protocols available

The physician and medical team will also consider many factors about the patient, including age and if patients have any existing conditions that might affect their reaction to treatment.

Treatment is based on how well the subtype of lymphoma typically responds to initial therapy. It is called risk-adapted therapy. Risk refers to the risk of cancer not responding successfully to treatment – that it either won’t respond well (refractory) or will return after remission (relapsed).

Patients whose type of lymphoma is typically less responsive to therapies are considered higher than normal risk. These patients are generally given more intense therapies.

Patients with a type of lymphoma that is likely to respond to initial therapy will receive medicines that are likely to have fewer side effects. All patients get medicines and other therapies to help with side effects.

The pediatric oncologist will discuss treatment options with families.

Non-Hodgkin lymphoma tends to be aggressive in children. It is important that treatment begins as soon as possible.

Non-Hodgkin Lymphoma that Doesn’t Respond or Comes Back

Although significant progress has been made in the treatment of children with lymphoma, 10-30% continue to have refractory or recurrent disease.

But there are treatment options. Many doctors consider intensive chemotherapy with or without treatment with targeted therapy, followed by a hematopoietic cell transplant (also called bone marrow transplant or stem cell transplant).

The subtype of lymphoma is an important factor in making treatment decisions for relapsed disease.

Prognosis for Non-Hodgkin Lymphoma

The prognosis (chance of recovery) depends upon

  • Type of lymphoma
  • Where the tumor is in the body when diagnosed
  • Stage of the cancer
  • Whether there are particular changes in the chromosomes
  • Type of initial treatment
  • Whether the lymphoma responded to initial treatment
  • The age and general health of the patient

Overall five-year survival rates for childhood non-Hodgkin lymphoma can range from about 70% to more than 90%.

Late Effects of Non-Hodgkin Lymphoma Treatment

Late effects are side effects that develop after treatment is completed. These include:

Patients should discuss any concerns with their physicians and let them know about any symptoms. Regular, focused follow-up care is extremely important for childhood cancer survivors.

Focus of Current Non-Hodgkin Lymphoma Research

  • New targeted therapy strategies are currently under investigation.
  • Immunotherapy is under study to improve survival rates.
  • Research is focused on the reduction and elimination of late effects of therapy such as infertility, heart problems, and second cancers.
  • The continued identification of chromosomal abnormalities associated with childhood lymphoma may prove helpful in refining the classification of subtypes, evaluating response to therapy, and developing novel therapies that target the involved gene or protein directly.


Reviewed: June 2019