Non-Hodgkin Lymphoma (NHL): Comprehensive Health Information

Non-Hodgkin Lymphoma (NHL): Overview

General Information

Lymphoma is a general term for cancers that originate in the lymphatic system, a vital part of the body's immune system. Non-Hodgkin Lymphoma (NHL) specifically develops from a type of white blood cell called lymphocytes, which include both B-cells and T-cells. The disease may begin in the bone marrow, spleen, thymus, or lymph nodes and can then spread to other parts of the body.

NHL is a relatively common malignancy. Early diagnosis paired with modern, advanced treatment methods offers a promising outlook, with high survival rates. If the cancer is confined to a single area, the survival rate is approximately 83%. Even in its most advanced stages, the survival rate for Non-Hodgkin Lymphoma remains greater than 60%.

Components of the Lymphatic System

The lymphatic system is a crucial network that transports disease-fighting white blood cells throughout the body. It includes:

• Lymph: A fluid that carries lymphocytes through a network of lymph vessels, helping to fight against infection and cancer.
• Lymph Nodes: Tiny, bean-shaped masses located throughout the body (e.g., underarm, neck, groin, pelvis, and abdomen). They filter lymph and store white blood cells to help combat disease.
• Spleen: An organ on the left side of the abdomen that helps lymphocytes develop, stores blood cells, and removes old blood cells.

• Thymus: A small organ located in the chest that aids in the production and development of lymphocytes.
• Tonsils: Nodes at the back of the throat that store white blood cells.
• Bone Marrow: The soft material within bones that produces blood cells, including white blood cells.

Types and Classification of Non-Hodgkin Lymphoma

NHL is classified based on the type of lymphocyte involved (B-cell or T-cell) and the rate at which the cancer grows and spreads.

B-cell Lymphoma

This is the most common form of NHL, accounting for about 85% of cases.

T-cell Lymphoma

These types are generally less common and include:

Precursor T-lymphoblastic lymphoma/leukemia: A rare type that often starts behind the breastbone.
Peripheral T-cell lymphomas: A group of rare lymphomas derived from mature T-cells, including:
- Cutaneous T-cell lymphomas (CTCL): Lymphomas of the skin.
- Adult T-cell leukemia/lymphoma: Linked to the HTLV-1 virus.
- Angioimmunoblastic T-cell lymphoma: Makes up roughly 4% of all lymphomas and affects the lymph nodes, liver, or spleen.
- Extranodal natural killer/T-cell lymphoma: Typically found in the nose and upper throat.
- Enteropathy-associated intestinal T-cell lymphoma (EATL): Found in the digestive tract lining, with Type 1 linked to celiac disease and Type II being less common.
- Anaplastic large cell lymphoma (ALCL): A fast-growing type, categorised by subtype (Primary cutaneous, Systemic, and Implant-associated ALCL).
- Peripheral T-cell lymphomas, not otherwise specified: A category for T-cell lymphomas that do not fit into the other groups.

Classification by Growth Rate:

Low-grade (Indolent) Non-Hodgkin Lymphoma: Develops slowly. Includes Marginal zone lymphoma, Mucosa-associated lymphoid tissue (MALT) lymphoma, Follicular lymphoma, and Mantle cell lymphoma.
Intermediate grade Non-Hodgkin Lymphoma: Includes Diffuse large cell lymphoma, Primary mediastinal large cell lymphoma, and Anaplastic large cell lymphoma.
High-grade Non-Hodgkin Lymphoma: Grows quickly. Includes Burkitt's lymphoma and Lymphoblastic lymphoma.

Additional Definitions:

Relapsed Non-Hodgkin Lymphoma: The disease returns after a patient has been successfully treated.
Refractory Non-Hodgkin Lymphoma: New or relapsed disease that does not respond to treatment.

Risk Factors

While the exact cause of NHL is not fully understood, certain factors can increase the likelihood of developing the condition:

• Gender: It is slightly more common in men.
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• Environmental Exposure: Some studies suggest a potential link between living in a farming community and exposure to certain herbicides and pesticides, though this is not definitively proven.

• Infections: Certain bacteria or viruses, including Human Immunodeficiency Virus (HIV), Epstein-Barr Virus (EBV), Human T-lymphotropic Virus (HTLV), and the bacterium Helicobacter pylori.
• Hereditary Cancer Syndromes.

If you have concerns about any of these risk factors, it is important to discuss them with your doctor.

Symptoms

Non-Hodgkin Lymphoma symptoms can vary significantly between individuals. Common symptoms may include:

• Painless swelling of lymph nodes in the neck, groin, or underarm
• Fevers or heavy night sweats
• Tiredness or fatigue
• Unexplained weight loss
• Severe itchiness

• Reddened patches on the skin
• Nausea, vomiting, or abdominal pain
• Coughing or shortness of breath
• Headaches, difficulty concentrating, or personality changes

Symptoms by Disease Grade:

Low-grade (Indolent) NHL

Develops slowly. Patients may primarily notice painless swelling of lymph nodes (often in the neck or over the collarbone) but otherwise appear healthy. Swelling may resolve and return. If the lymphoma spreads outside the lymph nodes, discomfort may occur in the affected area.

Aggressive NHL

Grows more quickly and generally presents with more pronounced symptoms, which can include:

Pain in the neck, arms, or abdomen
Fever and/or night sweats
Unexplained weight loss
Shortness of breath
Weakness in arms and/or legs
Confusion

Please note that these symptoms are not exclusively linked to Non-Hodgkin Lymphoma. It is essential to discuss any new or persistent symptoms with your doctor as they may signal other health issues.

Diagnosis

Accurate and precise diagnosis of Non-Hodgkin Lymphoma is critical for doctors to determine the most effective treatment plan and is key to successful treatment.

At SSCHRC, our team of experts includes specialised doctors called hematopathologists who focus on diagnosing lymphoma and other blood cancers. Using modern equipment and a high level of expertise, they determine the precise extent of the disease, which makes a significant difference in accurate diagnosis and successful treatment.

Non-Hodgkin Lymphoma Diagnostic Tests

If a patient presents with symptoms that suggest NHL, their doctor will conduct an examination and ask about their medical history. One or more of the following tests may be used to confirm a diagnosis, determine if the cancer has spread, and monitor treatment efficacy:

Lymph Node Biopsy

A small piece of tissue, or sometimes the entire lymph node, is surgically removed and examined under a microscope.

Imaging Tests

Used to visualise the body and check for signs of cancer spread, including:

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

Other Diagnostic Tests

Blood Tests: Used to check if blood cells are normal in number and appearance and to assess blood chemistry. Specific blood tests may also help doctors determine the patient's outlook.
Bone Marrow Aspiration and Biopsy.
Liver and Kidney Function Tests.
Echocardiogram: An evaluation of the size and function of the heart.
Immunophenotyping: Cells from a lymph node, blood, or bone marrow are examined to identify the specific type of Non-Hodgkin Lymphoma cells present.
Pulmonary Function Test: Measures how effectively the lungs are functioning.

Treatment

SSCHRC is dedicated to helping people with Non-Hodgkin Lymphoma achieve longer, healthier lives through advanced therapies designed to minimise side effects. Our Lymphoma and Myeloma Centre has been instrumental in shaping how lymphoma is treated globally.

The optimal treatment for NHL is personalised and depends on several factors:

• The specific type of lymphoma.
• The stage and category of the disease.

• The patient's symptoms.
• The patient's age and overall health.

Since there are over 60 different types of lymphoma, each treatment approach is customised to the patient's particular needs. One or more of the following therapies may be recommended:

Chemotherapy

This is the treatment most frequently used for NHL. It works by killing fast-growing cells, including cancer cells. Advanced methods like liposomal drug delivery may be used to enhance effectiveness. Because chemotherapy can lower certain blood cell counts, a transfusion of blood cell growth factors may sometimes be necessary.

Radiation Therapy

Uses focused beams of energy to destroy cancer cells. It may be used for early-stage lymphoma or to help relieve symptoms like pain, but it is rarely the only treatment given.

Immunotherapy

Drugs that work by helping the body's own immune system to fight the cancer, often resulting in fewer side effects than other treatments. Immunotherapy for NHL may include:

Chimeric Antigen Receptor (CAR) T cell therapy.
Monoclonal antibodies, such as rituximab.
Biological therapies that develop antibodies to help the body fight the cancer.
Immune modulators, such as lenalidomide, which modify the tumour cell environment to allow the immune system to kill the cancer.
Targeted therapies that use small molecules to block pathways cancer cells use to survive and multiply.
Small molecule treatments.

Stem Cell Transplantation

This procedure may be recommended if NHL does not respond to initial chemotherapy or if it returns. It replaces defective or damaged cells in patients whose normal blood cells have been affected by cancer or destroyed by high-dose chemotherapy.

Watchful Waiting

This approach involves closely monitoring the Non-Hodgkin Lymphoma without starting active treatment immediately. It is sometimes an appropriate strategy for certain patients with low-grade (slow-growing) lymphomas.