From discovery to differentiation: why subtypes matter in lymphoma

First described by Italian physician Marcello Malpighi as 'a disease of lymph nodes and spleen that was uniformly fatal',1 lymphoma was formally described by British physician Thomas Hodgkin in 1832.2 Beyond Hodgkin’s disease, other types of lymphoma, collectively known as non-Hodgkin lymphomas (NHL), have been identified.3

A group of different blood cancers that start in organs such as the lymph nodes, NHLs have key traits in common: they affect white blood cells known as lymphocytes and impair the body’s immune system over time.5

Development and use of classification systems has helped provide doctors and researchers with a framework to better understand NHL and has ultimately led to better outcomes for patients.

Classified into two distinct categories, depending on how fast or slowly the cancer grows, NHL can be either aggressive (fast-growing) or indolent (slow-growing).3

Accounting for 30% of new NHL diagnoses,7 DLBCL occurs primarily in older people and requires prompt treatment due to its aggressive nature. The first sign is usually enlarged lymph nodes, but it can also affect organs outside of the lymphatic system and is characterised by symptoms including unexplained weight loss, fever and night sweats.

Follicular lymphoma accounts for approximately 20% of new diagnoses of NHL.7 Due to its slow-growing nature, many of the symptoms often only appear in later stages of the disease.8 As a result, it can spread unnoticed in the body, causing eight out of ten patients to have advanced stage disease at the time of diagnosis.9

When treating patients with DLBCL, ensuring that the disease never returns is the ultimate goal.9 However, in as many as 40% of patients, DLBCL can return – at which point controlling the disease becomes the focus of treatment.10

For patients with follicular lymphoma, treatment goals include keeping the disease under control while maintaining a good quality of life. Although follicular lymphoma is generally considered incurable, treatment choices are improving and patients can remain in treatment-free remission for long periods of time.11

Given the multitude of subtypes within lymphoma, continued research and understanding of this complex disease will ensure patients receive the best possible treatment.

References

1. Cowan DH. Vera Peters and the curability of Hodgkin disease. Current Oncology. 2008; 15(5): 206-210.

2. Hodgkin T. On Some Morbid Appearances of the absorbent glands and spleen. 1832.

3. Lymphoma Research Foundation. About lymphoma. [Internet; cited April 2022]. Available at:

4. Globocan 2020. World Fact Sheet. [Internet; cited April 2022]. Available at:

5. American Society of Hematology. Lymphoma. [Internet; cited April 2022]. Available at:

6. Swerdlow SH, et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. In: World Health Organization Classification of Tumours. Lyon, France: IARC; 2017.

7. Cancer.Net. Leukemia – Lymphoma –Non-Hodgkin: Subtype. [Internet; cited April 2022]. Available from: :

8. National Institute for Health and Clinical Excellence Review of TA 110:rituximab for the first-line treatment of stage III-IV follicular lymphoma. [Internet; cited April 2022]. Available at:

9. Lymphoma Action. Diffuse large B-cell lymphoma. [Internet; cited April 2022]. Available at:

10. Maurer, JM et al. (2014). Event-free survival at 24 months is a robust end point for disease-related outcome in diffuse large B-cell lymphoma treated with immunochemotherapy. J Clin Oncol 32: 1066-73.

11. Fowler N. Role of Maintenance Rituximab (Rituxan) Therapy In the Treatment of Follicular Lymphoma. Pharmacy and Therapeutics; 2011; 36:590-598.