Lymphoma In Children: Types, Causes, Diagnosis & Treatment

Lymphoma, a type of cancer, develops in the lymphatic system, consisting of the lymph nodes, lymph vessels, thymus, spleen, and bone marrow (1). It occurs when lymphocytes (a type of white blood cell) grow abnormally and uncontrollably (2). The disease often spreads to the entire lymphatic system before detection (3). Sometimes, it also spreads to the lungs, brain, bone, and bone marrow.

Lymphoma is the third most common cancer after leukemia and brain tumor in children aged 0–14 (4). The risk of having the disease is more than double in boys (2).

Read the post to learn about the types, causes, risks, diagnosis, and treatment options of lymphoma in children.

What Are The Types Of Lymphoma In Children?

Lymphomas in children are of different types, which are divided into two major groups (2) (5):

1. Hodgkin lymphoma: These lymphomas have large, bi-nucleated cells called Reed-Sternberg cells. Hodgkin lymphoma is further divided into subtypes based on the cancerous cells’ appearance. However, most Hodgkin lymphomas are treated similarly. 

The most common symptom of Hodgkin lymphoma is painless inflammation of the lymph nodes in the neck, armpit, groin, or above the collarbone.

2. Non-Hodgkin lymphoma (NHL): These lymphomas are rare before the age of three but commonly seen in children under 15.

NHL is divided into subtypes based on the lymphocytes that show malignancy (cancerous growth). The three major NHLs that occur in children are (3):

• Lymphoblastic lymphoma: It originates in T-cells and accounts for around 30% of childhood NHL.
• Burkitt lymphoma: It originates from B-cells and accounts for around 40–50% of childhood NHL.
• Large cell lymphoma: It originates from either B-cells or T-cells and accounts for 20–25% of childhood NHL.

Non-Hodgkin lymphoma most commonly occurs in children with AIDS (3).

Types of lymphoma in children

Source: Lymphoma in children

How Common Is Lymphoma In Children?

In the US, around 800 cases of childhood lymphoma are diagnosed every year (3). Furthermore, around 8% of cancers affecting children and teens are lymphoma: 5% have non-Hodgkin lymphoma, while 3% have Hodgkin lymphoma (6).

NHLs are commonly seen in children aged zero to 14, while children above 19 have an equal possibility of developing Hodgkin lymphoma or non-Hodgkin lymphoma. That said, it is uncommon for a child under three years to have lymphoma. Additionally, lymphoma is more commonly seen in Caucasians than in African-Americans (6). In the US, 800 cases of childhood lymphoma are diagnosed every year (3).

What Are The Causes And Risk Factors Of Lymphoma In Children?

The exact cause for lymphoma in children is unknown; however, viral infections, weak immune systems, and genetic factors may develop the disease. Some common risk factors could be (7) (8).

• Human immunodeficiency virus—the AIDS-causing virus
• Radiation therapy for the treatment of cancer
• Congenital immune system conditions
• Family history of the disease
• Immuno-suppressive drugs taken after an organ transplant
• The Epstein-Barr virus (it causes some types of Hodgkin lymphoma and Burkitt lymphoma)

In most cases, the disease occurs due to genetic mutations of growing blood cells. Sometimes, regular pediatric checkups can help in detecting the disease early (5).

What Are The Symptoms Of Lymphoma In Children?

The symptoms of lymphoma vary greatly among children. Some may not experience any major symptoms except swollen lymph nodes, while others may experience multiple symptoms. These are some of the common symptoms of lymphoma in children (2) (3) (7):

• Painless swollen lymph nodes in the neck, underarms, or groin
• Extreme fatigue
• Fever without known reasons
• Weight loss
• Itching
• Night sweats
• Shortness of breath
• Poor appetite, abdominal pain, or diarrhea
• Difficulty in swallowing
• Facial swelling
• Chest pain 

What Are The Complications Of Lymphoma In Children?

The complications of lymphoma depend on the type and the stage of the disease. Some of the major complications experienced by children are (7).

• Lung problems
• Heart disease and blood vessel damage
• Changes in learning and thinking
• Memory loss
• Increased risk of other types of cancer later in life
• Delayed growth and development
• Death

How Is Lymphoma Diagnosed In Children?

First, healthcare providers will do a complete physical checkup to examine the size of the lymph node(s). They will also check the abdomen, chest, and other organs for fluid build-up (2) (5).

If the healthcare providers suspect that the lymph node enlargement is due to an infection, they will prescribe an antibiotic. However, if the lymph node does not shrink, further tests are done.

1. Biopsy, where a small amount of the fluid, tissue sample, or cell is removed from the enlarged node and tested. This procedure is done under general anesthesia. Sometimes, the entire lymph node is removed during a biopsy. Based on the biopsy results, further tests are recommended.

2. An X-ray or scan is recommended when lymph nodes are deep seated and cannot be felt outside the body.

3. If lymphoma is confirmed in the child, some of the following tests are done to confirm the stage of the disease (5):

• Blood tests
• Computer tomography scan (CT scan)
• Positron-emission tomography scan (PET scan)
• Magnetic resonance imaging (MRI)
• Bone marrow biopsy (a needle inserted into the hip bone collects the bone marrow) and lumbar puncture(cerebrospinal fluid is collected from the spinal column)
• Liver and kidney function tests

These tests help specialists understand the disease in detail and decide the treatment.

What Is The Treatment For Lymphoma In Children?

Generally, four treatment options are available. Your child might get one or a combination of these treatments (2).

1. Chemotherapy: Different chemotherapy drugs called cytotoxic drugs are available, from which your doctor will decide the suitable one for your child. 

It might take several months to complete all the cycles of chemotherapy. However, the duration of the therapy depends on the severity of the disease.

2. Radiotherapy: Only some children with Hodgkin lymphoma require radiotherapy, which is administered after chemotherapy. In this process, powerful X-rays are used to kill the cancer cells. Each radiotherapy session is only a few minutes long, but the entire treatment may take several days or weeks.

3. Surgery: Surgery is uncommon in children. However, in cases where the lymphoma cells grow slowly, surgery can be opted in the initial stages. Children who undergo surgery without other cancer treatments are closely monitored for signs of relapse.

What Are The Short-Term And Long-Term Side Effects Of The Treatment?

Lymphoma treatment kills some healthy cells along with the diseased cells. Therefore, with the removal of the disease, children experience other side effects too, most of which are short term, such as (2)

• Nausea and vomiting
• Loss of hair
• Increased risk of infection due to neutropenia (low levels of a type of white blood cell)
• Fatigue and shortness of breath due to anemia (low levels of red blood cells)
• Higher bruising and bleeding tendencies due to thrombocytopenia (low level of platelets)
• Weight loss or gain
• Sore mouth

Some long-term side effects are

• Fertility issues
• Risk of heart disease and stroke
• Risk of developing other cancers
• Lung problems
• Soft bones and tissues

Generally, children recover well without long-term side effects. However, the results differ among children.

What Is The Survival Rate Of Children With Lymphoma?

The survival rate depends on the seriousness of the disease (6).

• In Hodgkin lymphoma, the survival rate is between 90% and 95% five years after diagnosis if the disease is diagnosed early. If the disease has spread, the survival rate is 90% five years after diagnosis.

• In non-Hodgkin lymphoma, 80% of children stay disease-free five years after diagnosis. If cancer has not spread, the survival rate is 90% five years after diagnosis.

After Treatment

After the children’s treatment is completed, doctors check to ensure no evidence of lymphoma is left in the body. Children will then have regular follow-up tests for many years to ensure the lymphoma does not recur. With time, the chances of cancer recurrence decrease (2).

If you see any unusual changes in your child, consult a specialist since the sooner the disease is detected, the better are the chances of remission. The available treatment options are constantly improving, as are the survival rates. Discuss with your specialist to decide the best option for your child.

References

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Reviewer

Dr. Salla SemmaneMD

 

Dr. Salla Semmane is a practicing pediatrician in Rabat in the district of Hassan, Morocco. Born in Finland, she completed her medical studies at the Faculty of Medicine in Kuopio and had her Licentiate of Medicine in 1989.

Read full bio of Dr. Salla Semmane