Understanding Lymphoma

Renowned culinary expert, Datuk Dr. Redzuawan Ismail or better known as Chef Wan is a prominent celebrity chef and culinary icon in Malaysia. Recently in the latest headlines, he disclosed his recent cancer diagnosis in an Instagram update on 3rd August 2023. Through the post, the 65-year-old chef shared that he received the unfortunate news of having lymphoma cancer after undergoing a lymph node biopsy. Lucky for him, since the cancer was being discovered relatively early, the chances of him being cured were as 70 - 80%.

What is Lymphoma

Lymphoma is a type of cancer that originates in the lymphatic system, which is a part of the body’s immune system. The lymphatic system is made up of lymph nodes, lymph vessels as well as lymphoid tissues such as the spleen and tonsils. Lymphoma occurs when there is an abnormal growth of lymphocytes, a type of white blood cell that helps our body to fight off infections. If these abnormal lymphocytes continue to form and build up, they can spread through the lymph vessels to form a tumour in another part of the lymphatic system. 

Occasionally, lymphoma may also travel through the bloodstream to form a tumour in an organ outside the lymphatic system, such as the liver or the lungs. This will subsequently lead to main organ dysfunction and health deterioration, and possibly death if not treated early. 

Generally, there are 2 main types of lymphoma:

1. Hodgkin lymphoma (HL) also sometimes known as Hodgkin’s disease, is a blood cancer that begins in the white blood cells called lymphocytes. The disease usually starts in a lymph node at one or more places in the body, often first noticed in the neck. It can spread through the lymphatic system from one group of lymph nodes to another, and to other lymph tissue, especially the spleen and bone marrow. Though rare, Hodgkin lymphoma may spread outside the lymphatic system to form tumors in other organs such as the liver or lungs, a condition known as extranodal disease.

2. Non-Hodgkin lymphoma (NHL)
Ranked as the 8th new cancer case diagnosed in 2020 and attributed to 4 in every 100 patients newly diagnosed with cancer, NHL typically develops from B and T lymphocytes in the lymph nodes or tissues throughout the body. Tumor growth in the NHL may not affect every lymph node, often found skipping some and growing on other organs. NHL can be further subdivided into:
(i) Slow growing (low grade or indolent)
(ii) Fast growing (high grade or aggressive)

What causes lymphoma?

Like most cancers, the majority of the genetic mutations that cause lymphoma happen spontaneously, without an identifiable cause. But research suggests the following conditions or issues that may increase your risk of developing lymphoma include:

1. Certain viruses 
2. Infection with Epstein-Barr virus (glandular fever) or human immunodeficiency virus (HIV) has an increased risk of getting lymphoma, but this explains only a small number of Hodgkin lymphoma cases.
3. Family history
   Having a parent, brother, or sister who has had Hodgkin lymphoma slightly increases the risk of developing lymphoma though this family link is uncommon. 
4. Weakened immune system
   People whose immune system is not working as well as it should are more predisposed to lymphoma. Instances where immunity may be compromised:
   - taking immunosuppressive drugs to stop organ rejection following a transplant
   - HIV and/or AIDS
   - being born with rare medical conditions that affect the immunity including autoimmune diseases
5. Exposure to toxic agents such as pesticide, herbicides, benzene

All these aside, it is important to note that many people with known risk factors don’t necessarily develop Hodgkin lymphoma, and most people who do get it have no risk factors. Hodgkin lymphoma is also not contagious in general.

How do I know if I have lymphoma? 

Symptoms suggestive of Hodgkin or non-Hodgkin lymphoma include: 

• Painless swelling of one or more lymph nodes in the neck, armpits, or groin that doesn’t go away within a few weeks of treatment 
• Persistent fatigue, despite getting adequate hours of sleep
• Prolonged or recurrent fever (body temperature higher than 39.5 degrees)
• Shortness of breath
• Unexplained weight loss where patients would lose as much as 10% of total body weight over 6 months without dieting or exercise.
• Excessive night sweats
• Itchy skin

How do doctors diagnose lymphoma?

1. Blood tests: A full blood count (FBC) will be done to check the number of white blood cells and this may also include a lactate dehydrogenase level. Both elevation in these parameters would signal the possibility of lymphoma. 
2. Lymph node biopsy: A process where the enlarged lymph node will be removed, either partially or entirely and observed under a microscope. If the swollen lymph node is situated close to the skin’s surface, the whole lymph node will be removed, a procedure known as excision biopsy. It is usually done under local or general anesthesia, often as an outpatient procedure. On the other hand, if the enlarged lymph node is deeper in the body, a tissue sample will be obtained using a needle with the use of an ultrasound or CT scan. This procedure is referred to as an ultrasound or CT guided core biopsy and is generally done under local anesthesia within the radiology department. 
3. Imaging procedures: A chest X-ray or CT scan of the chest or neck may aid in identifying the existence of tumors or additional swollen lymph nodes, thus indicating the stage of lymphoma. Positron Emission Tomography (PET) scanning is a more advanced technique employed to detect lymphoma. 
4. Bone marrow biopsy: Since lymphoma is associated with the bone marrow, a bone marrow biopsy would be helpful in establishing the spread of the disease. This generally involves the insertion of a needle into the bone to obtain the bone marrow. In adults, the most common site of biopsy is the pelvic done and it is usually performed under local anesthesia or sedation.

Treatment options available

Lymphoma treatment involves a variety of approaches depending on the type, stage, and individual factors of the disease, which the course of treatment is dependent on. Indolent, or slow growing lymphoma may not need treatment as watchful waiting alone may be enough to ensure the cancer does not spread.

Essentially, the goal of treatment is to eliminate or control the cancer cells while also minimizing side effects and maintaining the patient's quality of life. If treatment is needed, it may involve the following: 

1. Biologic therapy: This is a drug treatment that activates the immune system’s response against cancer by introducing living microorganisms into the body
2. Antibody therapy: Synthetic antibodies are introduced into the bloodstream by a medical professional. These antibodies are thought to recognize and respond to the cancer’s toxins.
3. Chemotherapy: Involves aggressive drug treatment administered by a healthcare team to selectively target and eliminate cancer cells.
4. Radioimmunotherapy: This method uses the delivery of potent radioactive doses directly to the cancerous B and T-cells, resulting in their destruction.
5. Radiation therapy: Your doctor may recommend this type of therapy to target and destroy small areas where the lymphoma has targeted. High doses of radiation are employed to target and eradicate localized cancer areas.
6. Stem cell transplantation: This can help restore damaged bone marrow following intensive chemotherapy or radiation therapy.
7. Surgery: While the removal of organs such as the spleen might be suggested when the lymphoma is found spreading, the primary purpose of surgery is usually to obtain a biopsy instead. 

What are the survival rates for lymphoma?

The 5 year survival rate for non-Hodgkin lymphoma accounts for about 69% whereas for Hodgkin’s lymphoma, the survival rate is 91-94% for stage 1 & 2, 59-90% for stage 3 & 4. Thus, consistent and lifelong monitoring remains crucial, even upon treatment completion. It’s noteworthy that certain lymphomas could experience a recurrence despite completing treatment and may then need chemotherapy and/or stem cell transplant. 

In a nutshell

Lymphoma, as a type of cancer, is a serious and chronic illness. Fortunately, lymphoma treatments continue to improve, extending the survival rates for most. It is also evident that prompt, aggressive treatment is key as it can prolong a person’s life and may cure the cancer. 

This article is written by Janelle Leong, Bpharm(Hons) (DOC2US),
reviewed by Dr. Ahmad Haniffan, MBBS​ (DOC2US)

REFERENCES

1. http://www.myhealth.gov.my/en/lymphoma-primeyears/ 
2. https://www.cancer.org.au/assets/pdf/understanding-hodgkin-lymphoma-booklet#_ga=2.43247206.2101179085.1691503242-1320056854.1691503242 
3. https://www.medicalnewstoday.com/articles/146136 
4. https://my.clevelandclinic.org/health/diseases/22225-lymphoma 
5. https://www.webmd.com/cancer/lymphoma/lymphoma-cancer#1-5

Written by

Janelle Leong, Bpharm(Hons) DOC2US