Childhood Lymphoma: Comprehensive Cancer Information

Childhood Lymphoma: A Comprehensive Guide

We understand that a diagnosis of lymphoma for a child can be deeply concerning. This information is designed to provide you with a clear, empathetic, and medically accurate overview of childhood lymphoma, covering its nature, signs, diagnosis, and treatment options.

General Information

What is Lymphoma?

Lymphoma is a form of cancer that originates in the lymphatic system, a vital part of the body's immune system. The lymphatic system is responsible for producing, storing, and carrying immune cells, called lymphocytes, throughout the body.

Lymphoma develops when a lymphocyte undergoes a mutation during its development. This abnormal cell then begins to multiply rapidly, ultimately crowding out and displacing healthy immune system cells.

Types of Lymphocytes Involved

T cells

These cells assist the immune system in responding to disease and are directly responsible for killing abnormal cells.

B cells

These cells produce antibodies, which are specialised proteins that recognise and attack foreign substances like bacteria and viruses.

Parts of the Lymphatic System

The lymphatic system is made up of several interconnected components, including:

• Lymph: A clear fluid that carries lymphocytes through a network of lymph vessels (similar to tiny veins). Lymph is crucial for fighting infection and cancer.
• Lymph Nodes: These are tiny, bean-shaped masses found in various parts of the body, such as the neck, underarm, groin, abdomen, and pelvis. They filter the lymph fluid and store white blood cells to help the body fight disease.
• Spleen: An organ located on the left side of the abdomen that aids in the development of lymphocytes, stores blood cells, and removes old blood cells.

• Thymus: A small organ in the chest that is essential for making and developing lymphocytes.
• Tonsils: Nodes at the back of the throat that store white blood cells.
• Bone Marrow: The spongy material inside bones that produces all types of blood cells, including white blood cells like lymphocytes.

Lymphoma Classification

Lymphoma is broadly categorised based on how the cancer cells appear when examined under a microscope: Hodgkin Lymphoma and Non-Hodgkin Lymphoma.

Hodgkin Lymphoma (HL)

Key Feature: Almost all cases of Hodgkin Lymphoma are characterised by the presence of large, abnormal cells called Reed-Sternberg cells, which often have more than one nucleus. These cells grow, divide, and survive longer than normal cells.
Subtypes: The vast majority of cases are known as classical Hodgkin Lymphoma.
Prognosis: The five-year overall survival rate for Hodgkin Lymphoma patients under the age of 20 is approximately 98%.

Non-Hodgkin Lymphoma (NHL)

Key Feature: Non-Hodgkin Lymphoma does not contain Reed-Sternberg cells.
Subtypes: NHL has several subtypes, including:
- Burkitt's Lymphoma (BL): Affects B cell lymphocytes and is recognised as one of the fastest-growing cancers.
- Lymphoblastic Lymphoma (LBL): Primarily affects T cell lymphocytes and is similar to Acute Lymphoblastic Leukaemia (ALL). It is more common in boys.
- Large Cell Lymphoma (LCL): Includes Diffuse Large B-cell Lymphoma (DLBCL), which typically affects pre-adolescents and teenagers, and Anaplastic Large Cell Lymphoma (ALCL), which is more common in adolescents.
Progression: Most paediatric NHL cases are an aggressive form of the disease.
Prognosis: Despite its aggressive nature, the five-year survival rate for children and adolescents with NHL is about 90%.

Childhood Lymphoma Risk Factors

A risk factor is anything that increases a person's chances of developing a disease. While many paediatric lymphoma patients do not have any known risk factors, those associated with the disease include:

• Sex: Hodgkin Lymphoma is more common in males than in females.
• Infections: Including the Epstein-Barr virus (which causes mononucleosis) and HIV.
• Immune System Diseases: Such as lupus and rheumatoid arthritis.

• Immune Deficiency Syndromes: Including Ataxia-telangiectasia, Bloom syndrome, Common variable immunodeficiency, Severe Combined Immunodeficiency Syndrome (SCID), and Wiskott-Aldrich syndrome.
• Medical History: Taking immunosuppressant drugs following an organ transplant.
• Family History: Having a parent or sibling with the disease. Some cases of lymphoma can be passed down genetically, and genetic counselling may be recommended.

Symptoms

Lymphoma symptoms can vary significantly from person to person, and some individuals may not notice symptoms for an extended period. Recognising potential warning signs is critical for early diagnosis and effective treatment.

Early Signs and Symptoms

These are often the first, more subtle indications of lymphoma:

• Painless swelling of lymph nodes in the neck, underarm, or groin.
• Persistent fatigue that does not improve with rest.
• Unexplained fevers.
• Heavy night sweats (B symptoms).

• Loss of appetite.
• Unexplained weight loss (B symptoms).
• Itchy, dry skin or a red rash.

Late Signs and Symptoms

As the disease advances, symptoms may become more severe or widespread:

• Nausea, vomiting, or abdominal pain.
• A feeling of fullness in the abdomen, often caused by an enlarged liver or spleen.
• Coughing or shortness of breath, typically due to enlarged lymph nodes in the chest.

• Headaches.
• Difficulty concentrating and confusion (neurological symptoms).
• Weakness in the arms and/or legs (neurological symptoms).

What Lymphoma May Feel Like

The most common physical sign of lymphoma is a painless swelling of the lymph nodes, most often in the neck, armpit, or groin. These nodes may feel firm or rubbery. Many patients also experience deep, unrelenting fatigue, unexplained fevers, unexplained weight loss, and drenching night sweats.

Symptoms can be specific to the cancer's location:

In the chest, it may cause chest pain, a persistent cough, or difficulty breathing.
In the abdomen, it can lead to swelling, pain, or a feeling of fullness.

It is crucial to note that these symptoms can also be caused by other, non-cancerous conditions.

Other Conditions with Similar Symptoms

Symptoms of lymphoma can overlap with other conditions, including:

• Viral infections (e.g., mononucleosis or HIV).
• Autoimmune disorders (e.g., lupus or rheumatoid arthritis).
• Tuberculosis or other chronic infections.

• Other cancers, such as leukaemia or solid tumours.
• Skin conditions (e.g., eczema or psoriasis) for rash and itching.
• Thyroid disorders (for weight changes and fatigue).

When to See a Doctor

It is recommended to consult a healthcare provider if you or your child experiences:

Persistent or unexplained swelling of lymph nodes.
Fever, night sweats, or weight loss that does not improve.
Ongoing fatigue or shortness of breath.
New or worsening neurological symptoms (confusion, weakness).
Any combination of the above symptoms lasting more than two weeks.

Diagnosis

An accurate and precise diagnosis is essential for doctors to select the most effective course of treatment.

At SSCHRC, our expert group includes highly specialised doctors called hematopathologists, who focus on diagnosing lymphoma and other blood cancers. They utilise modern equipment to determine the exact extent of the disease, which significantly contributes to accurate diagnosis and successful treatment planning.

Lymphoma Diagnostic Tests

If a child exhibits symptoms that could signal lymphoma, a doctor will perform a medical examination and gather information on the family's medical history. One or more of the following procedures may be used to confirm the presence of cancer, determine if it has spread (staging), and monitor treatment effectiveness:

Lymph Node Biopsy

The entire lymph node or a sample piece is removed and examined under a microscope.

Imaging Tests

These non-invasive procedures provide detailed pictures of the body, and may include:

X-rays
CT or CAT (Computed Axial Tomography) scans
PET (Positron Emission Tomography) scans
MRI (Magnetic Resonance Imaging) scans

Blood Tests

Conducted to check if blood cells are normal in number and appearance, and to assess blood chemistry.

Bone Marrow Aspiration and Biopsy

A long needle is used to remove a sample of bone marrow, typically from the pelvic bone, for examination.

Liver and Kidney Function Tests

These tests can assist in both the diagnosis and the determination of prognosis.

Echocardiogram and Pulmonary Function Tests

Performed before treatment begins to assess how well the heart (echocardiogram) and lungs (pulmonary function tests) are working. This information helps doctors determine the intensity of treatment a patient can safely receive.

Immunophenotyping

Tests carried out on blood or tissue samples to pinpoint the exact type of lymphoma, which is vital for tailoring the optimal treatment plan.

Lymphoma Staging

Following a lymphoma diagnosis, various tests are performed to determine if cancer cells have spread within the lymph system or to other parts of the body. This process is called staging, and the stage is used by doctors to help plan treatment.

Childhood Hodgkin Lymphoma Stages (Source: National Cancer Institute)

Stage I: Cancer is found in one or more lymph nodes in a single group, or in rare cases, in the Waldeyer's ring, thymus, or spleen.
- Stage IE: Cancer has spread to one area outside the lymph system (e.g., an organ or tissue).
Stage II: Cancer is found in two or more lymph node groups, all located either above or all below the diaphragm.
- Stage IIE: Cancer has spread from a group of lymph nodes to a nearby organ outside the lymph system. It may also have spread to other lymph node groups on the same side of the diaphragm.
Stage III: Cancer is found in lymph node groups both above and below the diaphragm, or in lymph node groups above the diaphragm and in the spleen.
Stage IV: The cancer has spread extensively throughout one or more organs outside the lymph system, or it is found in multiple groups of lymph nodes on both sides of the diaphragm and in an organ outside the lymph system, or has spread to the lungs, liver, or bone marrow from distant areas.

Additional Notations (A, B, E, or S)

Letters may be used to further describe the stage:

A: The patient does not have B symptoms (fever, weight loss, or drenching night sweats).
B: The patient does have B symptoms.
E: Cancer is found in an organ or tissue outside the lymph system, which may be next to an area of the lymph system already affected by cancer.
S: Cancer is found in the spleen.

Childhood Non-Hodgkin Lymphoma Stages (Source: National Cancer Institute)

Stage I: Cancer is found in one group of lymph nodes, or in one area outside the lymph nodes. No cancer is found in the abdomen or the area between the lungs (mediastinum).
Stage II: Cancer is found in one area outside the lymph nodes and in nearby lymph nodes; or in two or more areas either above or below the diaphragm; or started in the stomach or intestines and can be completely removed by surgery (with possible spread to nearby lymph nodes).
Stage III: Cancer is found in at least one area above the diaphragm and at least one area below the diaphragm; or started in the chest; or started in the abdomen and spread widely, making complete surgical removal impossible; or is found in the area around the spine.
Stage IV: Cancer is found in the bone marrow, brain, or cerebrospinal fluid. Cancer may also be found in other parts of the body.

Treatment

At SSCHRC, we are dedicated to providing customised care for your child's lymphoma. Our experts utilise the most advanced treatments and techniques, aiming to minimise the impact on your child's developing body. Our extensive experience in these cancers can significantly improve your child's outcome.

Core Treatment Options

Chemotherapy

Chemotherapy drugs are used to kill cancer cells, control their growth, or relieve disease-related symptoms. Because the lymph system carries fluid throughout the body, allowing lymphoma to spread easily, chemotherapy—which also travels throughout the body—is the most common treatment. It may involve a single drug or a combination of two or more agents.

Immunotherapy

Immunotherapy is designed to enhance the immune system's natural ability to find and eliminate cancer cells. This is crucial because cancer can often evade the immune system.

Specific immunotherapies used include:

Immune Checkpoint Inhibitors: These drugs prevent the immune system from prematurely 'turning off,' allowing cancer-fighting immune cells (T cells) to mount a stronger, more sustained response against the disease.
CAR T cell therapy: T cells are modified so they can specifically recognise and attack cancer cells. This therapy is currently used to treat various types of lymphoma through clinical trials and is approved for certain B-cell lymphoma subtypes (a type of non-Hodgkin lymphoma).

Targeted Therapy

Targeted therapy drugs are designed to interfere with or "target" specific molecules (often proteins) that cancer cells need to survive, multiply, and spread. By working at a cellular level, these drugs aim to stop or slow the cancer's progression.

Stem Cell Transplantation

Also known as a bone marrow transplant, this procedure replaces cancerous bone marrow with new, healthy bone marrow stem cells. For paediatric lymphoma, the healthy stem cells can be sourced from a donor or from the patient. Patients typically receive this transplant after an intense round of chemotherapy.

Radiation Therapy

Radiation therapy uses powerful, focused beams of energy to destroy cancer cells while minimising damage to surrounding healthy tissue. It is sometimes used alongside chemotherapy to treat large affected areas or to relieve discomfort caused by cancerous lymph nodes pressing on nearby organs. While a primary treatment for adult lymphoma, doctors are limiting its use in children due to the risk of long-term side effects on a growing body.

Surgery

Surgery may be performed in paediatric lymphoma patients when tumours cause severe and immediate problems, such as intestinal blockages, intense pain from compressed nerves or organs, or breathing difficulties. In rare instances, it may be used to help achieve a cure.