Lymphoma in Children by Dr. Stalin Ramprakash

1. Lymph

This is a colourless fluid that acts as a carrier of proteins, nutrients, and fats from the digestive system. It also transports T cells and B cells to the blood.

2. Lymph vessels

Lymph vessels extend throughout the body. They carry lymph. Basically, these tiny vessels carry the tissue fluid back to the central circulation.

3. Spleen

Spleen is an organ that stores many lymphocytes and destroys the old blood cells thus acting as a blood filter. It plays a major role in the immune system and fights against infections.

4. Lymph nodes

Lymph nodes are the small bean-shaped organs found across the lymph vessels. They store white blood cells required to fight various infections. They play a major role in the early recognition of invading microorganisms and initiating the immune response.

5. Thymus

The thymus is an organ that is present in the upper part of the chest behind the chest bone. It is an organ in which the T lymphocytes are stored, educated and mature into immuno-competent T cells.

T lymphocytes or T cells are the type of cells that acts as a part of antigen-specific immunity. Cancers that are associated with the lymphatic system or lymphatic organs in children are called pediatric Lymphomas). The prognosis (chance of recovery) depends on the type of and stage of the cancer.

Causes of Lymphoma in Children

The cause for lymphoma in the majority of children remains unknown. Possible risk factors include

1. Certain long-term medical treatments related to immunity.
2. Certain viral infections like HIV and Epstein- Barr virus.
3. Previous treatments for cancers, include radiation.
4. Treatments that include immunosuppressive therapy.
5. Inborn defects in the immune system.
6. Treatments that include organ transplants.

Any lymphatic disease or Lymphoma that follows certain conditions like viral diseases like HIV, immunosuppressive therapies, and organ transplants are referred to as associated lymphoproliferative disease.

What Are the Types of Lymphoma?

There are two main types of childhood lymphoma.

1. Hodkin Lymphoma

HL or Hodgkin Lymphoma is of two major types.

a) Classical Hodgkin Lymphoma

Classical Hodgkin Lymphoma can be identified by the presence of Reed-Sternberg cells among the affected lymphocytes (lymphocytes are a type of white blood cell). Reed-Sternberg cells are the affected lymphocytes that have turned into cancerous cells that are seen with multiple nuclei.

b) Nodular Lymphocyte Predominant Hodgkin Lymphoma or NLPHL

Nodular Lymphocyte Predominant Hodgkin Lymphoma is a rare type of lymphoma that occurs mostly in children younger than 10 years. Affected cancer cells are microscopically observed to find a popcorn-shaped cell that distinguishes NLPHL from Classic Hodgkin Lymphoma. This particular type is very slow-growing and has a very good prognosis.

2. Non-Hodgkin Lymphoma

NHL or Non-Hodgkin Lymphoma is of three major types in children

a) Aggressive Mature B Cell Non-Hodgkin Lymphoma

This type of Non-Hodgkin Lymphoma develops in B cells or B lymphocytes. It is again divided into three subtypes.

• Burkitt Lymphoma or Burkitt Leukaemia

This may develop in lymph nodes and then spread to blood and bone marrow. It is called leukaemia or lymphoma arbitrarily based on how much bone marrow is involved but the treatment approach remains the same. These are very aggressive tumours but outcomes are good if treated with very intense chemotherapy treatment protocols that are being used currently.

• Diffuse Large B Cell Lymphoma

This type of lymphoma develops in the B lymphocytes of the lymph nodes and may spread to other lymphatic organs like the spleen and is mostly seen in adolescents. It is a very quickly developing type of cancer and may further spread to the liver, bone marrow, and other organs of the body. Again, outcomes are good on the current chemotherapy treatment.

• Primary Mediastinal B Cell Lymphoma

This is the cancer of the mediastinum. The mediastinum represents the structures that are present behind the breastbone. This particular rare type usually occurs in older girls and young women. It often arises from the B lymphocytes present in the thymus in the mediastinum and can spread to the organs that are located nearby.

b) Lymphoblastic Lymphoma

Lymphoblastic lymphoma is a type of Non-Hodgkin Lymphoma that starts developing in immature T lymphocytes. T lymphocytes are the type of white blood cells that possess specific antigen immunity and are produced and multiplied in a lymphatic organ called the thymus. It is a type of cancer that can spread to the lymphatic and non-lymphatic organs.

c) Anaplastic Large Cell Lymphoma

Mutations in a particular type of gene called Anaplastic Lymphoma Kinase can cause this cancer. It develops in T lymphocytes and can spread to the other lymphatic organs and non-lymphatic organs.

[Read : Fanconi Anaemia in Children]

Risk Factors of Lymphoma in Children

There are no known risk factors that can cause lymphoma in a healthy child. A healthy immune system plays a crucial role in weeding out any cancerous cells at a very early stage before it becomes a problem and if the immune system is not functioning well, it may predispose the individual to develop cancers like lymphoma.

Certain factors that may trigger the development of lymphoma are

1. Previous Cancer Treatments And Radiation Exposure

One of the factors that may lead to the development of lymphoma is a compromised immune system. Previous cancer treatments like chemotherapy and radiation can weaken the immune system and induce mutations in the genes leading to secondary cancers like Lymphoma.

2. Viral Infections

Viral infections like Human Immuno Virus or HIV can weaken the immune system. The Epstein-Barr virus is another viral infection that is held responsible for causing genetic changes in the cell and leading to lymphoma in children.

3. Organ Transplant

An organ transplant may cause many complications. Due to the mediations and weakened immune system post-transplant might count as one of the reasons.

4. Additional Factors

Other risk factors include inherited defective DNA syndromes. Such conditions can cause gene mutations thus triggering lymphoma.

Signs and Symptoms of Lymphoma in Kids

Symptoms of pediatric lymphoma include

• Persistent unexplained fever in spite of reasonable investigations and treatment lasting for more than 2-3 weeks
• Painless swollen lymph nodes in the neck, groin, and armpits which are slowly increasing in size over weeks or few months.
• Trouble breathing and trouble swallowing.
• Wheezing sounds while breathing especially when lying flat.
• Extreme night sweats that are drenching.
• Swollen neck, and head.
• Swollen lump in the abdominal area.
• Fatigue and weight loss.
• Decreased appetite.
• Painless lump in testicles.

Contact your child’s paediatrician if you notice any of the above symptoms.

How is Lymphoma Diagnosed?

Doctors may prescribe certain tests to confirm if your child has lymphoma.

1. Blood Tests and Blood Chemistry

Doctors may require a complete blood picture and blood chemistry study through which they can identify the signs of abnormality. Liver functioning test, the amount of uric acid, creatinine, and electrolyte levels may give some clues in identifying the presence of disease.

2. Physical Examination

Doctors may conduct a physical examination to notice if there is any abnormal swelling in the lymph nodes. They may also ask for the clinical history of the child. This can help in finding the right path for the diagnosis and the treatment. Prognosis may also depend on the child’s pathology reports and pre-existing medical conditions.

3. MRI (Magnetic Resonance Imaging)

An MRI gives detailed images of the body parts. It will be done by passing the child into a large machine that uses magnetic and radio waves to give the images. Doctors may require this to identify abnormal tumors inside the body.

4. CT Scan

A computed tomography scan of the whole body can provide detailed information of the body parts at all angles.

5. PET Scan

A PET scan or Positron Emission Tomography scan is conducted specially to diagnose the presence and locations of cancer areas. The child will be injected with the required amount of radioactive glucose into the body through IV. Cancer cells, as they multiply faster than the normal cells tend to consume more glucose and look brighter in the scan.

6. X-Ray

A chest X-Ray can provide clear pictures of the organs inside the chest region.

7. Biopsy

Children with lymphoma symptoms will need a biopsy to confirm the diagnosis. In case of swollen lymph nodes doctors may consider performing a core biopsy of the lymph nodes using interventional radiology techniques. Fine needle aspirations should be avoided as they can be falsely negative and often do not give adequate information to make a definitive diagnosis. The child may also be required to undergo a small surgery that includes the removal of a complete lymph node or a part of the lymph node for testing.

8. Bone Marrow Apsiration

A bone marrow aspiration will be done by inserting a fine needle into the hip bone to collect the sample from the bone marrow. Pathologists will test the sample for the identification of abnormal cells.

9. Mediastinoscopy

Your child may require other tests like mediastinoscopy if there is any abnormal tumor found in the mediastinum. This is a small surgical procedure that requires an incision that may help to look into the organs in the mediastinum.

10. Histopathology

Other than histopathology (Looking at the cells under microscope) often the pathologist will need to perform special staining of the cells (Immunohistochemistry) to correctly identify the type of the cancer cells.

Further tests will be prescribed once the cancer is confirmed.

Stages of Lymphoma in Children

Lymphoma has four stages like all the other cancers.

Stages I and II

Stage I and stage II are considered to be early stages and the prognosis can be more if the cancer is diagnosed at these stages. In stage I, cancer will be limited to one lymph node or the group of lymph nodes in the same area and not out of that particular area. In stage II the lymphoma can be found in one area with local lymph node involvement or two areas on the same side of the diaphragm or an intestinal tumour resected completely. Such stages respond quickly and well to the treatment provided, and the prognosis will be above 90%.

Stages III and IV

Stage III and stage IV are considered to be advanced stages where the prognosis depending on the type of cancer and the stage and still can be above 75-80%. In stage III, cancer will be found on either side of the diaphragm or incompletely resected intestinal tumour. In stage IV, the lymphoma spreads to the central nervous system or bone marrow. Prognosis also depends on the child’s response to the advanced treatments given hence there will be a reassessment of tumour response after a few cycles of chemotherapy.

Treatments Available For Lymphoma in Children

There are treatments available for lymphoma in children. A pediatric oncologist, after deciding the stage and the advancement of cancer may discuss the right treatment options with you. Some of the standard treatments include.

1. Chemotherapy

Medications called chemotherapy are used to kill cancer cells. Chemotherapy is the mainstay of treatment for lymphomas. This helps in inhibiting the rapid multiplication of cancer cells. Advanced stages of cancer that reach the brain and cerebrospinal fluid may need intrathecal chemotherapy (Injecting medications directly into the cerebrospinal fluid). This can treat cancer that has already spread to the brain, or this may help avoid spreading cancer to the brain and cerebrospinal fluid. The intensity of the chemotherapy and the number of cycles will depend on the type and stage of the lymphoma.

2. Radiation

Radiation therapy is inducing high-energy radiation to kill cancer cells. This can lead to a considerable amount of side effects on the child. Radiotherapy is particularly used in Hodgkin’s lymphoma if there was a suboptimal response to chemotherapy during the reassessment after initial chemotherapy.

3. High Dose Chemotherapy

High-dose chemotherapy includes drugs that are high in dosage to kill the cancer cells. High-dose chemotherapy is administered in the case of a relapsed lymphoma or an advanced stage lymphoma. Here the patient’s own stem cell will be collected before administration of high dose chemotherapy and re-infused after the chemotherapy. The process is otherwise known as autologous stem cell transplant or high dose chemotherapy followed by autologous stem cell rescue.

4. Immunotherapy

Immunotherapy is a type of treatment where the immunological treatment that targets the cancer cells (eg. Rituximab – monoclonal antibody against B cells, CAR -T cell therapy- T cells are engineered to recognise T cells and attack them).

5. Targeted Therapy

This is a kind of treatment where only certain specific cancer cells will be targeted and killed. Different types of lymphomas require different targeted therapy treatments.

[Read : Bone Marrow Transplant For Children]

Side Effects of Treatment For Lymphoma in a Child

The treatments administered for the type of lymphoma may cause certain side effects like

• Nausea
• Hair loss
• Mouth ulcers
• Low blood counts needing blood and platelet transfusions
• Low white cell counts resulting in fever and need for antibiotic treatment
• Weakness
• Secondary cancers
• Poor learning abilities
• Loss of appetite
• Infertility

Chances of Recurrence of Lymphoma in Kids

Lymphoma in children is a completely curable disease and has seen a good prognosis. But, there is no guarantee that cancer will not return after the treatment. Children who recover from lymphoma need to get frequent medical tests to monitor their recurrence. Children with successful treatments can have a prolonged cancer-free life.

Other Treatment Options Available After the Recurrence of Lymphoma in Children

If there is a recurrence of the lymphoma in children, doctors may plan for advanced treatments like

• High dose chemotherapy along with stem cell transplant can help in killing the cancer cells effectively.
• Involving your child in clinical trials like immunotherapy and targeted therapy can help. Targeted therapy works only on the cells that are affected and cause lesser side effects comparatively.
• Radiations if the lymphoma has affected a single part of the body.

Lymphoma in children can be an extremely overwhelming situation to handle for parents and caregivers. Consulting your child’s pediatric oncologist at the earliest notice of any obvious symptom can help in dealing with the situation without complications. It is important to support your child mentally and physically during such times. Childhood lymphoma is completely curable if diagnosed early. There are many treatment options available and the prognosis is very good in children. After your child’s remission, it is important to get their health checkups done in regular intervals of time to make sure that there is no relapse.

FAQ’s

1. How Long Can a Child Live With Lymphoma?

The survival rate of children with lymphoma after the treatment with early diagnosis is about 90%.

2. What Are the Signs of Lymphoma in Children?

Lymphoma in children surface symptoms like

• Swollen lymph nodes in the neck, armpit, and groin.
• Drenching night sweats.
• Fever for an unknown reason.
• Breathing problem and trouble swallowing.
• Wheezing sounds while breathing.
• Swollen neck or swollen head.
• Painless lump in the abdominal area.
• Weight Loss and loss of appetite.

3. What is The Most Common Type of Lymphoma in Children?

There are two types of lymphomas, Hodgkin Lymphoma and Non-Hodgkin Lymphoma. According to a study, Non-Hodgkin lymphoma is the most common type of lymphoma in children.

4. Can Children Recover From Lymphoma?

The chance of recovery in children with lymphoma is very high with the treatment options available with the medical advancements nowadays. A child can fully recover from lymphoma and can stay in remission for a very long period.

Read Also: Leukaemia in Children – Symptoms, Diagnosis and Treatment by Dr. Stalin Ramprakash