Mantle-cell lymphoma (MCL) usually responds well to initial treatment, but most patients eventually relapse or become refractory to treatment. The term relapsed refers to disease that reappears or grows again after a period of remission. The term refractory is used to describe when the lymphoma does not respond to treatment (meaning that the cancer cells continue to grow) or when the response to treatment does not last very long.1
For patients who relapse or become refractory, secondary therapies may be successful in providing another remission. Like other forms of non-Hodgkin lymphoma, no consensus has been reached regarding the best treatment for relapsed or refractory MCL. Fortunately, however, an increasing number of treatment options are available for these patients. The type of treatment recommended for any individual patient depends on several factors, including the timing of the relapse, the patient’s age, extent of disease, overall health, and prior therapies received.2
Sometimes lymphoma specialists prescribe chemotherapy-based treatments for patients whose MCL has relapsed or become refractory. In addition, several agents have been approved by the US Food and Drug Administration (FDA) for the treatment of relapsed or refractory MCL, including2,3:
- Acalabrutinib (Calquence)
- Bortezomib (Velcade) with or without rituximab
- Ibrutinib (Imbruvica)
- Lenalidomide (Revlimid) with or without rituximab.
These treatment options work differently than chemotherapy and are called targeted therapies. Bortezomib, lenalidomide, and ibrutinib have all been available for several years to treat other hematologic cancers, and their use has been expanded to also treat relapsed or refractory MCL.2,3 Acalabrutinib is a newer treatment that was approved by the FDA in October 2017.4
A number of other drugs to treat MCL are being studied in clinical trials. Some studies are evaluating new combinations of drugs, whereas others are investigating entirely chemotherapy-free regimens in the relapsed/refractory and frontline settings. These drugs include venetoclax (Venclexta), idelalisib (Zydelig), carfilzomib (Kyprolis), daratumumab (Darzalex), buparlisib, and the CAR T-cell therapy, axicabtagene ciloleucel (Yescarta).3
Opportunities may exist for patients with MCL to join a clinical trial evaluating new therapies. If interested, patients should speak with their hematologist about participating in such a trial.
References
- National Organization for Rare Disorders. Mantle cell lymphoma. https://rarediseases.org/rare-diseases/mantle-cell-lymphoma. Accessed April 17, 2019.
- Lymphoma Research Foundation. Mantle cell lymphoma: relapsed/refractory. www.lymphoma.org/aboutlymphoma/nhl/mcl/relapsedmcl. Accessed April 17, 2019.
- Parrott M, Rule S, Kelleher M, Wilson J. A systematic review of treatments of relapsed/refractory mantle cell lymphoma. Clin Lymphoma Myeloma Leuk. 2018;18:13-25.
- Calquence [prescribing information]. Wilmington, DE: AstraZeneca Pharmaceuticals LP; 2017.