Rare Lymphoma Subtypes: Raising Awareness
Lymphoma is the most common form of blood cancer and serves as an umbrella term for over 80 subtypes of cancer originating in the lymphatic system. The lymphatic system is a vital part of the immune system, helping to fight off infections and harmful cells in the body. Lymphoma occurs when lymphocytes— a type of white blood cell—grow and multiply uncontrollably.
Each subtype of lymphoma has its own clinical course, including distinct symptoms, diagnostic tests, staging (extent and spread of lymphoma), prognosis (outcome), treatment options, and follow-up care. Understanding your specific lymphoma subtype is important to ensure you receive the most appropriate treatment and information about your disease.
Most types of lymphomas have similar and widely recognized symptoms, such as swollen lymph nodes, fatigue, fever, loss of appetite, and coughing. However, some lymphoma subtypes are rare and may not be recognized or diagnosed promptly due to their uncommon or unfamiliar manifestations in the body. These rarer subtypes can take longer to diagnose and are often identified at more advanced stages because their symptoms may be mistaken for other disorders or diseases. Raising awareness about these rare subtypes is essential for earlier recognition and diagnosis.
Some examples of rarer lymphoma subtypes include cutaneous T-cell lymphoma (CTCL), Epstein-Barr virus (EBV) associated lymphoma, peripheral T-cell lymphoma (PTCL), extranodal NK/T-cell lymphoma, nasal type, and central nervous system (CNS) lymphoma. These represent just a few of the many rare subtypes of lymphoma.
Let’s look at the symptoms of some of these rarer subtypes:
Cutaneous T-cell Lymphoma (CTCL)
Cutaneous T-cell lymphoma presents itself with rashes, lumps, and redness on the skin, along with itchiness. CTCL is considered a slow-growing skin lymphoma that has many subtypes, the most common being mycosis fungoides (MF). It is often misdiagnosed as eczema or psoriasis, due to a lack of awareness that lymphoma can manifest on the skin. CTCL is diagnosed in approximately 340-370 individuals per year. Highest incidence rates were found in men and individuals older than 40. Treatment, after the correct diagnosis, is difficult to access in Canada as it is a rare cancer with limited resources.
Visit our CTCL subtype factsheet for more information: LymphomaCanada_FactSheet_CTCL_Digital.pdf
For more information and visual images on how CTCL can present on the skin, view this video: https://www.youtube.com/watch?v=3l80idC_xUY
Epstein-Barr virus (EBV) – Associated Lymphoma
The Epstein-Barr virus (EBV) is a highly contagious infection that can be spread through bodily fluids, such as saliva. Many people carry the EBV but remain asymptomatic and may not know they are infected. It is believed that EBV infects and persists in B lymphocytes, potentially leading to lymphoma in a small percentage of individuals. The reasons why some people with EBV develop lymphoma while others do not remain unknown.
Not everyone infected with EBV will develop cancer, as this is rare. Symptoms associated with EBV infection may include a sore throat, lack of appetite, swollen glands, weakness, and an enlarged spleen or liver. EBV-associated lymphoma is estimated to have approximately 1,199 cases per year
Peripheral T-cell lymphoma (PTCL)
Peripheral T-cell lymphoma (PTCL) is a rare and aggressive blood cancer that can affect multiple areas of the body, including the lymph nodes, skin, gastrointestinal tract, liver, and spleen. Many PTCL cases are associated with the Epstein-Barr Virus (EBV). Common symptoms of PTCL include a rash, abdominal pain, swollen lymph nodes, fever, fatigue, and enlargement of the liver and spleen. PTCL has an incidence rate of approximately 815 cases per year. It most commonly affects individuals over the age of 60 and is diagnosed slightly more often in men than in women. However, PTCL can also occur in children and younger adults.
Visit our PTCL subtypes factsheet for more information: LymphomaCanada_FactSheet_PTCL_Digital.pdf
Extranodal NK/T-cell lymphoma, nasal type lymphoma
Extranodal NK/T-cell lymphoma, nasal type, affects approximately 23 to 47 individuals per year in Canada, highlighting its rarity. This subtype commonly presents with symptoms such as nasal blockage, nasal bleeding, swelling, blocked airways, difficulty swallowing, and voice hoarseness. It is a subtype of Peripheral T-cell lymphoma (PTCL) and is strongly associated with the Epstein-Barr Virus (EBV). Extranodal NK/T-cell lymphoma, nasal type, is most commonly observed in individuals living in Asia and parts of South America. Additionally, other subtypes of extranodal NK/T-cell lymphoma can affect areas such as the skin, groin, and gastrointestinal tract.
Central nervous system lymphoma (CNS)
Central nervous system (CNS) lymphoma is an extremely rare subtype of lymphoma, with an incidence rate of approximately 200 cases per year. The CNS consists of the brain and spinal cord, and CNS lymphoma can form in these areas as well as in the spinal fluid. Additionally, CNS lymphoma can affect the eyes, a condition referred to as intraocular lymphoma.
Common symptoms of CNS lymphoma include weakness, vision deficits, hearing loss, seizures, mental fog, back pain, and difficulty with speech. Symptoms can change rapidly if the lymphoma spreads further into the CNS, as this system is critical for regulating many of the body’s essential functions and can impair an individual’s abilities. People with weakened immune systems—such as those diagnosed with HIV/AIDS or individuals who have undergone organ transplantation—are at an increased risk of developing primary CNS lymphoma. However, it is important to note that this type of lymphoma can also occur in individuals with healthy immune systems. CNS lymphoma is most commonly found in males over the age of 60.
If you are experiencing any of the following symptoms, talk to your doctor and raise any concerns you may have.
By raising awareness, Lymphoma Canada aims to reduce diagnostic delays, improve patient outcomes, and create a stronger support network for individuals affected by these lesser-known subtypes. Providing information to patients, caregivers, healthcare professionals, and the general public can lead to earlier diagnoses and timely, effective treatments, ultimately improving outcomes.
To access Lymphoma Canada’s subtype factsheets, visit: https://www.lymphoma.ca/lymphoma-subtype/
Join our monthly virtual support group: Lymphoma Canada Wellspring Support Group – Lymphoma Canada
To access our one-on-one peer mentoring program, visit: https://www.lymphoma.ca/resources/support/peer-support-program/
References
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Ghazawi, F. M., Netchiporouk, E., Rahme, E., Tsang, M., Moreau, L., Glassman, S., Provost, N., Gilbert, M., Jean, S., Pehr, K., Sasseville, D., & Litvinov, I. V. (2017). Comprehensive analysis of cutaneous t‐cell lymphoma (CTCL) incidence and mortality in Canada reveals changing trends and geographic clustering for this malignancy. Cancer 123, 3550–3567. https://doi.org/10.1002/cncr.30758
Green, K. (2023). Central Nervous System Lymphoma. StatPearls [Internet]. https://ncbi.nlm.nih.gov/books/NBK545145/
Jones, V. (2023). Central Nervous System (CNS) lymphoma: What you need to know. MD Anderson Cancer Center. https://www.mdanderson.org/cancerwise/central-nervous-system–cns–lymphoma–what-you-need-to-know.h00-159621012.html
Mayo Foundation for Medical Education and Research. (2023). Cutaneous T-cell lymphoma. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/cutaneous-t-cell-lymphoma/symptoms-causes/syc-20351056
Peripheral T-cell lymphoma. Leukemia & Lymphoma Society. (n.d.). https://www.lls.org/sites/default/files/2021-05/FS25_PTCL_1_21.pdf
Professional, C. C. Medical. (n.d.). Cutaneous T-cell lymphoma: Types, symptoms & prognosis. Cleveland Clinic. https://my.clevelandclinic.org/health/diseases/17940-cutaneous-t-cell-lymphoma
Professional, C. C. Medical. (n.d.). Peripheral T-cell lymphomas. Cleveland Clinic. https://my.clevelandclinic.org/health/diseases/25103-peripheral-t-cell-lymphoma
Professional, C. C. Medical. (n.d.). CNS lymphoma: Symptoms, prognosis & treatment. Cleveland Clinic. https://my.clevelandclinic.org/health/diseases/23474-cns-lymphoma
Richard., & Penny. (2024). Central Nervous System (CNS) lymphoma. Lymphoma Action. https://lymphoma-action.org.uk/types-lymphoma/central-nervous-system-cns-lymphoma
Shen, J., Luo, M., Liu, L., Chen, Q.-Y., Tang, L.-Q., & Mai, H.-Q. (2021). Incidence and trend of Epstein-Barr virus-related cancer: A surveillance, epidemiology, and end results program based study. Journal of Clinical Oncology 39, 10575–10575. https://doi.org/10.1200/jco.2021.39.15_suppl.10575
The link between Epstein-Barr virus and lymphoma. Lymphoma Action. (2023). https://lymphoma-action.org.uk/link-between-epstein-barr-virus-and-lymphoma
Thida, A. M. (2023). Extranodal NK-cell lymphoma. StatPearls [Internet]. https://www.ncbi.nlm.nih.gov/books/NBK559207/
Van Doesum, J. A., Niezink, A. G., Huls, G. A., Beijert, M., Diepstra, A., & van Meerten, T. (2021). Extranodal Natural Killer/T-cell lymphoma, nasal type: Diagnosis and treatment. HemaSphere, 5. https://doi.org/10.1097/hs9.0000000000000523
Watson, S., & Begum, J. (2023). Epstein-Barr virus (EBV): Causes, symptoms, and treatment. WebMD. https://www.webmd.com/a-to-z-guides/epstein-barr-virus
