Current treatment options and future treatment directions for mantle cell lymphoma

This review outlined the current treatment options for patients with mantle cell lymphoma and highlighted possible future therapies. 

Mantle cell lymphoma (MCL) is a type of non-Hodgkin lymphoma that usually affects older people and men. MCL is considered aggressive and incurable. MCL treatment is based on many factors, including age and disease status. Younger patients are generally treated with more aggressive therapies. Older patients (65 years or older) are often treated with less aggressive therapies because many of the side effects may outweigh the treatment benefits. 

Younger patients: Younger patients are better able to tolerate intensive treatment than older patients. This may also lead to longer periods of remission. Intensive treatments aimed at increasing the progression free survival (PFS; time from treatment to disease progression) time is the standard of care.

R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) is a commonly used chemotherapy combination. One treatment that has had good long term results is dose-intensified R-CHOP (maxi-CHOP) alternating with R + HiDAC (rituximab plus high-dose cytarabine) for 6 cycles followed by autologous stem cell transplantation (auto-SCT; using stem cells from the patient’s own body). One study followed patients for an average of 11.6 years. The average PFS was 8.5 years. The average overall survival (time from treatment to death from any cause) was 12.7 years.

Other similar high-intensity treatments also have good results. In another study, 60 patients received 3 cycles of R-CHOP followed by 3 cycles of R-DHAP (rituximab plus dexamethasone, HiDAC, and cisplatin) before auto-SCT. The average PFS was 7 years. The 5-year overall survival rate was 75%.

A study has shown that patients treated with rituximab maintenance therapy after intensive treatment have significantly better PFS and overall survival rates than patients who were observed only.

Older patients: Older patients are generally treated with much less aggressive therapy than younger patients. The combination of bendamustine and rituximab (BR) has been shown to have increased survival over other therapies. In a study of 94 patients with MCL, 46 received BR and 48 received R-CHOP. The average PFS for the BR patients was 35.4 months. The average PFS for patients in the R-CHOP group was 22.1 months. BR also had fewer side effects than other common MCL treatments.

Current studies are exploring ways to improve BR outcomes for older patients. One study added cytarabine (another chemotherapy medication) to the BR regimen, now called R-BAC. Newly diagnosed patients treated with R-BAC had a 2 year PFS rate of 95%. Patients with relapsed MCL had a 2-year PFS rate of 70%. More research is needed about R-BAC and other novel treatments.

If bendamustine or BR is not an option, VR-CAP bortezomib, rituximab, cyclophosphamide, doxorubicin, and prednisone) is a good therapy as well. In a study of 487 patients, 244 received R-CHOP and 243 received VR-CAP. The average follow-up time was 40 months. The average PFS for patients in the R-CHOP group was 14.4 months. The average PFS for patients in the VR-CAP group was 24.7 months. While PFS is improved with VR-CAP, the treatment has more side effects than R-CHOP.

Rituximab maintenance therapy has been shown to significantly improve PFS in patients who received R-CHOP. More research is needed on the long term outcomes of patients treated with maintenance rituximab after BR treatment.

Relapsed patients: There are several new treatment options for relapsed or refractory (difficult to treat) MCL. These treatments include bortezomib (Velcade), lenalidomide (Revlimid, alone or in combination with rituximab), temsirolimus (Torisel), ibrutinib (Imbruvica), Idelalisib (Zydelig), and venetoclax (Venclexta). 

All of these new treatments have been studied for their safety and effectiveness.

New treatments: Several ongoing studies are looking at new treatment regimens for both newly diagnosed patients and patients with relapsed or refractory disease. Some of these new formulations for older patients include the addition of some of the new treatments to the BR regimen. The new treatments being studied with BR include ibrutinib, lenalidomide, and bortezomib.

One of the new treatments for younger patients include the addition of ibrutinib to R-CHOP/R-DHAP plus auto-SCT treatment. 

This study reviewed the current treatment methods for MCL along with future experimental therapies currently being studied. 

All treatment decisions should be discussed with your doctor. Different treatment methods or medications may be more effective in some people than others. It is important to talk to your doctor about the different risks and benefits associated with each treatment strategy.