Lymphoma

Lymphoma is the most common type of blood cancer and originates from abnormal lymphocytes, a key component of the human immune system. There are two main types — Hodgkin lymphoma and non-Hodgkin lymphoma. Hodgkin lymphoma and non-Hodgkin lymphoma can occur in children, teens and adults of any age.^1-3

Hodgkin Lymphoma

According to the Lymphoma Coalition, more than 62.000 people worldwide are diagnosed with Hodgkin lymphoma each year, including about 300 in Belgium.. The disease occurs more frequently at younger age (<40) but can also occur at higher age (in general > 55).  The diagnosis process includes a series of tests to decipher the disease stage which can range from Stage I to Stage IV. In Stage I, only one lymph node region or single organ is affected. As the stages progress, more lymph node regions are affected, and in Stage IV, multiple organs and lymph node regions above and below the diaphragm are affected.⁴

Hodgkin lymphoma treatment generally consists of combination chemotherapy plus/minus radiotherapy. After initial treatment, the majority of Hodgkin lymphoma patients, in particular the younger patients will be cured, but some patients may require additional combination therapy, immunotherapy and/or stem cell transplantation.⁵

Non-Hodgkin Lymphoma

More than 60 non-Hodgkin lymphoma subtypes have been identified and assigned names by the World Health Organization (WHO), characterized by appearance, anatomical localization and cell composition.^6-9

These subtypes of non-Hodgkin lymphomas are divided into two major groups: T-cell lymphomas, which develop from abnormal T-lymphocytes, or B-cell lymphomas, which develop from abnormal B-lymphocytes. There are many different forms of T-cell lymphomas, some of which are extremely rare. T-cell lymphomas can be aggressive (fast- growing) or indolent (slow-growing).^6-9

There is no one-size-fits-all approach to treating non- Hodgkin lymphoma. Subtypes can vary so much that they present as a practically different disease altogether, so treatment options also vary greatly.¹⁰

Peripheral T-Cell Lymphomas (PTCLs)

Peripheral T-cell lymphomas (PTCLs) comprise a group of more than 25 subtypes of non-Hodgkin lymphoma. PTCL makes up 10-24% of non-Hodgkin lymphoma cases in various regions.¹¹ The most commonly used treatment options are combination chemotherapy regimens or other multidrug regimens. After the use of combination therapies, some patients may benefit from autologous stem cell transplant (ASCT), however, many patients are unable to receive transplants because they are not fit enough.¹¹

Systemic Anaplastic Large Cell Lymphoma (sALCL)

Systemic anaplastic large cell lymphoma (sALCL) is a common subtype of PTCL, and patients

with sALCL are divided into two groups: anaplastic lymphoma kinase-positive (ALK+) and anaplastic lymphoma kinase-negative (ALK-) ALCL. ALK+ ALCL occurs in younger individuals and responds well to standard chemotherapy treatments, putting most patients into long-term remission.¹²

Most people with ALK- ALCL initially respond to treatment as well, but the disease is more likely to relapse. Initial treatment for sALCL is a combination chemotherapy regimen. However, relapse occurs in approximately 40-65% of patients after initial frontline therapy, and outcomes are poor among patients. In many cases, prescribers turn to combination therapies as a treatment option.¹²

Cutaneous T-Cell Lymphoma (CTCL)

Cutaneous T-cell Lymphoma (CTCL) most commonly affects the skin as one or multiple skin lesions but can impact each patient differently.

Most CTCL types are generally treatable, but not curable. Patients with CTCL can receive either skin-directed or systemic therapies, depending upon the stage of their disease. ¹³

References:

¹ Lymphoma Research Foundation. About Lymphoma. https://lymphoma.org/aboutlymphoma/  Accessed January 2021.
² Center for Disease Control and Prevention. Lymphoma. https://www.cdc.gov/cancer/lymphoma/index.htm.  Accessed January 2021.  ³ Moffitt Cancer Center. Hodgkin & Non-Hodgkin Lymphomas. https://mofitt.org/cancers/lymphomas-hodgkin-and-non-hodgkin/ . Accessed January 2021.
⁴ American Cancer Society. Survival Rates for Hodgkin Lymphoma. https://www.cancer.org/cancer/hodgkin-lymphoma/detection-diagnosis-staging/staging.html  Accessed January 2021.
⁵ National Health Service (NHS) Hodgkin lymphoma treatment. https://www.nhs.uk/conditions/hodgkin-lymphoma/treatment/ . Accessed January 2021.
⁶ Leukemia & Lymphoma Society. Non-Hodgkin Lymphoma. https://www.lls.org/lymphoma/non-hodgkin-lymphoma . Accessed January 2021.
⁷ Swerdlow SH, et al. Blood 2016;127:2375-2390.
⁸ Vose J, et al. J Clin Oncol 2008;26(25):4124-4130.
⁹ Matutes E. Int J Lab Hem. 2018;40(1):97-103.
¹⁰ Leukemia & Lymphoma Society. Treatment. https://www.lls.org/lymphoma/non-hodgkin-lymphoma/treatment . Accessed August 2021.
¹¹ Leukemia & Lymphoma Society. Peripheral T-Cell Lymphoma Facts. https://www.lls.org/sites/default/files/file_assets/peripheraltcelllymphomafacts.pdf Accessed August 2021.
¹² Lymphoma Research Foundation. Understanding Anaplastic Large Cell Lymphoma. https://lymphoma.org/wp-content/uploads/2021/01/LRF-ALCL_Factsheet_2021.pdf. Accessed August 2021.
¹³ Lymphoma Research Foundation. Cutaneous T-Cell Lymphoma: Treatment Options. https://lymphoma.org/aboutlymphoma/nhl/ctcl/ctcltreatment/  Accessed August 2021.