September 15 marked the eighth anniversary of World Lymphoma Awareness Day. The event aims to increase public awareness on lymphoma, in terms of symptoms of the disease, diagnosis, and treatment modalities.
THE incidence of lymphoma is increasing, with one million people worldwide living with lymphoma and almost 1,000 patients diagnosed every day.
In Malaysia, according to the National Cancer Registry 2007, lymphoma is the sixth most common cancer among Malaysians and the sixth most common cancer in males, whereas in females, it is the eighth most common cancer.
The Chinese were found to have a higher incidence rate of lymphoma in comparison to Malays and Indians.
Even though it is one of the most common cancers diagnosed, there is still low public awareness on lymphoma compared to other cancers such as breast and lung cancer. The following is some pertinent information on lymphoma as shared by consultant haematologist Datuk Dr Vijaya Sangkar Jaganathan.
What is lymphoma?
Lymphoma is the general term for cancer of the lymphatic system, and it is one of the more common cancers that is faced by doctors worldwide every year. The lymphatic system is comprised of lymphatic vessels/tract and lymph nodes in the neck, armpit, chest, abdomen and groin.
Functionally, the lymphatics sieve and remove excess fluids from the body and play an important role in the immune system. The core lymphoma pathology is abnormal white cells called lymphocytes, which become cancer cells, multiply, and accumulate or infiltrate the lymph nodes.
Lymphomas are divided into two categories: Hodgkin’s Disease (HD) and Non-Hodgkin’s Lymphoma (NHL).
The main differentiating feature is from the microscopic description from a biopsied tissue sample, thus explaining the mandatory lymph node biopsy for an accurate diagnosis.
Generally NHL is more common than HD. Worldwide, the incidence is reported to be increasing either due to earlier detection or an absolute rise in incidence.
The need to differentiate the pathology has therapeutic implications since HD and NHL are treated with different types of medications, and generally have variable outcomes.
The exact risk factors or etiology for the development of lymphoma is essentially unknown, but people with Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome, auto-immune disease and previous radiation exposure have a higher risk of developing this cancer.
Infections with Epstein Barr Virus have been noted to be a trigger factor in genetically prone persons. This however has not been consistently seen.
Some of the more common presenting clinical features in lymphoma patients are as follows:
- Enlarged and palpable lymph nodes, commonly in the neck, armpit and groin.
- Unexplained febrile illness.
- Loss of weight.
- Loss of appetite.
- Unexplained itch/pruritus.
- Chest tightness, swelling and breath difficulties.
The most important initial assessment is a thorough history and a complete physical examination, paying particular attention to other medical and surgical issues in the individual. Physical examination also aids in looking for the best possible site for lymph node biopsy, with minimal morbidities.
After a solid pathological diagnosis is achieved, the individual will have the following tests performed for completion of clinical staging and risk stratification:
- Blood test to determine organ function
- Imaging, commonly whole body CT scan and occasionally PET scan
- Bone marrow examination
The initial assessment will give the treating doctor a complete view of the disease, taking into account the stage of disease, co-morbidities, and prognostic factors. Such information is vital in designing a treatment plan.
One important lymphoma feature that is not used in many risk stratification guidelines is bulk of disease. The size of the lymph node does play a crucial role in determining treatment type, schedule and consolidation therapy.
Clinical trials are suggested to some eligible patients, but this pathway is not very well accepted in this country in comparison to many parts of the world where most of their patients are involved in ongoing clinical trials using newer modalities of treatment or therapeutic breakthrough.
However, in the past three to five years, many more of our patients have consented to be involved in clinical trials. This will enable doctors to understand the disease better and improve treatment outcomes.
The ultimate staging in lymphoma is not to be accepted as something dreadful, although we know that Stage I/II do better than III/IV; staging is used to determine the intensity of treatment and for consolidation therapy once treatment is completed.
Overview of treatment
Not all lymphomas need to be treated as soon as possible. This is true for low grade lymphoma such as limited stage follicular lymphoma. A small cohort of NHL can be treated with surgical and antibiotic therapy, but this is very infrequent.
Stage I or very limited stage Hodgkin’s lymphoma can be solely treated with involved-field radiotherapy. A large majority of patients would require treatment, and they are generally grouped as follows:
·Targeted therapy using monoclonal antibodies.
·Stem cell transplantation, usually using autologous cells as a measure to consolidate treatment after the initial intensive chemotherapy to optimise long term outcomes. (Autologous means using the patients own stem cells after the disease is controlled.)
No two lymphomas will behave in the same way and this is because of the different disease biology, thus making interim assessment vital. For patients who demonstrate first line or second line chemotherapy resistance, the long term outcome is generally poor. Salvage therapy is sometimes advocated and if they do show chemosensitivity, than allogeneic (using a sibling or a matched unrelated person) stem cell transplantation can be performed.
Despite this, a significant cohort can still relapse.
Cure is possible in lymphomas when it is detected at an early stage or in very limited stage disease such as Stage I-II non bulky disease. Certainly, with current modalities of treatment, cure is seen in limited stage Hodgkin’s lymphoma.
Low grade NHL is essentially non-curable, but adequate therapy can put a patient in long term remission. In comparison, high grade lymphomas are treated more aggressively, and in certain situations, upfront stem cell transplantation is advocated after a complete remission (a state of disease-free period) is achieved.
This decision is based on individual response, ie chemosensitivity, underlying medical fitness, level of tolerance to chemotherapy, and infection risk.
Can you prevent lymphoma?
Lymphoma is not a hereditary disease, and in general, nothing can be done to prevent you from getting this cancer as the cause or etiology is not clearly understood and it is probably related to underlying somatic mutation in a genetically prone person.
From his years of experience in managing lymphoma cases, Dr Vijaya highlighted some important points to consider when an individual is faced with lymphoma:
- Undergo complete assessment to get a good overall picture of disease.
- Be compliant to therapy.
- Do not take antioxidants during chemotherapy as this may technically protect cancer cells and make chemotherapy less effective.
- Increase fluid intake.
- Consume healthy and cooked food.
- Stay away from crowded areas to minimise infection risk.
- Do not consume herbal or any other traditional supplement as this may interact with conventional chemotherapy, making them either more toxic, or chemotherapy less effective.