Health Resources

Lymphoma (Children)

2024/05/30
What Is Lymphoma

​Lymphomas are a group of cancers originating from the lymphatic system, a crucial component of the body's immune defence against foreign substances, infections and diseases. These cancers arise when lymphocytes, a type of white blood cell, proliferate uncontrollably. Lymphomas are broadly classified into Hodgkin lymphoma (formely known as Hodgkin's disease) and non-Hodgkin lymphoma, each with distinct spread patterns, effects on the body and responses to treatment. Lymphoma is the third most common cancer in children.

Hodgkin Lymphoma

  • Hodgkin lymphoma is identified by the presence of abnormal Reed-Sternberg amidst lymphocytic infiltration. Epstein-Barr virus infection, autoimmunity and immunodeficiency are implicated factors in its development. It exhibits a bimodal age distribution, with early peaks in the mid to late 20s and a later peak post-50 in developed countries, while in developing countries, the early peak often occurs before adolescence.

    Childhood Hodgkin lymphoma types:

    • Classical Hodgkin lymphoma: Characterised by four subtypes discerned through microscopic examination of the cancer cells, including lymphocyte-rich, nodular sclerosis, mixed cellularity and lymphocyte-depleted variants.

    • Nodular lymphocyte-predominant Hodgkin lymphoma

Non-Hodgkin Lymphoma

More prevalent in children under 10 years, non-Hodgkin lymphoma encompasses three primary types: mature B-cell lymphoma, precursor lymphoid lymphoma and mature T-cell lymphoma.

Mature B-cell Lymphoma

Comprising about 40% of paediatric non-Hodgkin lymphoma cases1, this type typically presents with enlarged lymph nodes in the throat, neck or abdomen. It may be found in the bones or bone marrow. There are 3 major subtypes:

  • Burkitt's lymphoma (BL)
  • Diffuse large B-cell lymphoma (DLBCL)
  • Primary mediastinal B-cell lymphoma (PMBL)

It can affect the bones, bone marrow and the mediastinum, potentially spreading to the lungs, heart sac and distant organs such as the kidneys. It's more common in older adolescents.

Precursor Lymphoid Lymphoma

Making up about 30% of non-Hodgkin lymphoma in children1, this can manifest as swollen lymph nodes or a significant mediastinal mass.

When more than 25% of bone marrow comprises blasts, it is classified as acute lymphoblastic leukaemia (ALL).

Mature T-cell Lymphoma

While rare, mature T-cell lymphomas like anaplastic large cell lymphoma (ALCL) and peripheral T-cell lymphoma (PTCL) can involve lymph nodes, bones and organs. ALCL is responsible for about 10% of childhood non-Hodgkin lymphoma2 and may present with skin rashes or lumps, rapid growth and severe systemic inflammation. 

Source: 1Principles and Practice of Paediatric Oncology (7th Edition), 2American Cancer Society

Causes Of Lymphoma

The exact cause of lymphoma in children remains unknown. However, there are certain risk factors that increase susceptibility:

  • Immune system disorders: Children with congenital or acquired immune system deficiencies have a higher likelihood of developing lymphoma.
  • Family history: Having a sibling with lymphoma marginally increases the risk.
  • Epstein-Barr virus: Previous infection with this virus is associated with an increased risk of lymphoma.
  • Environmental factors: To date, no environmental toxins have been definitively linked to an increased risk of lymphoma in children.
Signs And Symptoms Of Lymphoma

Lymphoma can manifest in various forms, making early diagnosis challenging. Symptoms might not be evident until the disease has progressed significantly

Hodgkin lymphoma symptoms:

  • Commonly in adolescent males, Hodgkin lymphoma may present with:
  • Persistent fevers
  • Cervical lymph node enlargement
  • Night sweats and
  • Weight loss and reduced appetite

Non-Hodgkin lymphoma symptoms:

  • The symptoms of Non-Hodgkin lymphoma can vary widely, including:
  • Non-specific symptoms such as loss of appetite and weight
  • Abdominal pain and swelling
  • Skin lumps and rashes
  • Cough and shortness of breath

Indicators of possible lymphoma:

  • Fevers
  • Sudden loss of appetite and weight
  • Night sweats
  • Enlarged lymph nodes
  • Cough and shortness of breath
  • Fatigue

Due to these varied symptoms, lymphoma is often initially misdiagnosed. For example, a child with fever and cough might be treated for tuberculosis, or abdominal pain might be mistaken for constipation, delaying the correct diagnosis of lymphoma.

Diagnosis And Treatment Options For Lymphoma

Led by a paediatric oncologist, the team at NUH designs a comprehensive treatment plan, beginning with a detailed history and physical examination to understand the child's symptoms and their progression. 

Diagnosis test and procedures

Blood Tests

  • These include complete blood count (CBC), biochemistry and liver function tests.
  • Children with lymphoma often exhibit an inflammatory profile in their CBC, especially in types like Hodgkin lymphoma, ALCL, Burkitt's lymphoma (BL) and diffuse large B-cell lymphoma (DLBCL).
  • Inflammatory markers may show elevated leukocyte and neutrophils counts, high eosinophils, lymphopenia, increased platelet count or anaemia.

Imaging Studies

  • Chest X-ray
  • Computed tomography (CT) scans
  • Positron emission tomography (PET) scans, where a small amount of radioactive glucose is used to highlight malignant cells, which consume more glucose than normal cells.

Other Additional Tests

  • Lumbar puncture: To examine spinal fluid for central nervous system involvement.
  • Bone marrow aspiration and trephine biopsy: Sampling of bone marrow and a small piece of bone using a hollow needle, usually from the hipbone or breastbone.
  • Directed biopsy: Tissue samples are taken from the affected area, such as a rash, lump or lymph node.

Treatment Options

Chemotherapy

Chemotherapy, which is central to lymphoma treatment, employs powerful medications to destroy or inhibit the growth and division of cancer cells. These medications are typically administered intravenously, but some are oral.  Combination therapy, using various medications simultaneously, is common, with treatment duration depending on the cancer's type and stage.

For queries about chemotherapy and potential side effects such as hair loss and fertility issues, please consult your paediatric oncologist.

Radiation Therapy

Radiation therapy employs high-energy X-rays or other types of radiation to target cancer cells. In young children, it can affect bones and soft tissue growth and may increase the risk of heart disease and secondary cancers later in life. It is less commonly used in treating Hodgkin lymphoma in children.

Surgery

Lymphoma usually spreads throughout the lymph system, negating the presence of a specific tumour site. However, if there is a significant accumulation of lymphoma in one area, surgery to remove the tumour may be considered.

Targeted Therapy

Increasingly utilised for advanced or treatment-resistant lymphomas, targeted therapy attacks specific substances essential for cancer growth wile sparing normal cells. Examples include using Brentuximab, an anti-CD30 antibody-drug conjugate targeting CD30 on lymphoma cells, and Crizotinib, an anti-Anaplastic Lymphoma Kinase (ALK) inhibitor used in high-risk ALK-positive ALCL cases.

Crizotinib, an anti-Anaplastic Lymphoma Kinase (ALK) inhibitor, is used as upfront therapy to target ALK-positive ALCL for high-risk cases.

Stem Cell Transplant

For hard-to-treat lymphoma or recurrence after chemotherapy, high-dose chemotherapy followed by an autologous stem cell transplant (using the child's own stem cells) may be proposed. The child's stem cells are harvested, stored and later infused back into their body.

In certain situations, an allogeneic transplant (using a donor's stem cells) may be required, particularly if the child's lymphoma relapses or does not respond to an autologous transplant.

Follow-Up Care

Effective follow-up care is a vital aspect of treatment for children with lymphoma. The frequency of follow-up visits depends on the type of cancer and the treatments used. Children undergoing treatment may need follow-up checks as often as weekly or as infrequently as monthly.

After treatment completion, follow-up care continues. Typically, visits are scheduled every three months for the first year, biannually for the second year and then annually thereafter.

These visits may include blood tests and imaging tests like PET-CT scans to monitor for lymphoma recurrence. Additionally, late effects of cancer treatment will be assessed, including growth and hormone disturbances and organ function issues in the liver, kidneys, lungs and heart.

Clinical Outcomes

Hodgkin Lymphoma generally has a more favourable prognosis compared to non-Hodgkin Lymphoma. Non-Hodgkin lymphoma outcomes can vary significantly due to the diverse types and biological characteristics of the disease, impacting treatment response. The five-year survival rates for non-Hodgkin lymphoma range from 45–87%, while early-state Hodgkin's lymphoma sees survival rates around 95%3.

At 9a Viva-University Children's Cancer Centre, the five-year survival rate for treatment-naïve Hodgkin Lymphoma is 100%, and 88.5% for non-Hodgkin lymphoma. However, survival rates for relapsed patients, especially those with non-Hodgkin lymphoma, remain lower, indicating a need for further research in this area.

Information is correct as of September 2017.

Source: 3National Cancer Institute

Research On Lymphoma In Children

Continued clinical research is essential for advancing paediatric healthcare. Our doctors are committed to contributing to the future of child health and medicine through both clinical practice and research.

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