ESMO Guidelines for mantle cell lymphoma

This study presented guidelines for the treatment and follow-up of mantle cell lymphoma presented by the European Society for Medical Oncology. 

Mantle cell lymphoma (MCL) is a rare but usually aggressive type of non-Hodgkin lymphoma. Patients often experience multiple relapses after first- and second-line treatment. The treatment strategy for MCL depends on disease stage, whether or not the patient has already received treatment, and the patient’s age. 

This article outlined the European Society for Medical Oncology (ESMO) guidelines for the treatment and follow-up care for patients with mantle cell lymphoma.

First occurrence of MCL:

Stage I/II: Patients with newly diagnosed stage I or II MCL should be treated with chemotherapy followed by radiotherapy. Patients with stage I or II who have risk factors such as larger or a high number of tumors should be treated like advanced stage patients.

Stage III/IV: Patients with stage III or IV MCL should begin treatment as soon as possible after diagnosis. A watch-and-wait strategy is not recommended.

For elderly patients (over 65) with advanced stage disease, intensified chemotherapy is not an option. R-CHOP (rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone) is recommended for elderly patients. Other treatments being explored are high-dose cytarabine plus CHOP and R-BAC (rituximab plus bendamustine and cytarabine). Maintenance therapy with rituximab has been shown to significantly increase survival.

For younger patients (younger than 65) with advanced stage disease, a more intensive approach is recommended. R-DHAP (rituximab plus dexamethasone, cytarabine, and cisplatin) followed by rituximab maintenance therapy is the standard of care. Autologous stem cell transplantation (auto-SCT; using stem cells from the patient’s own body) is also part of the standard of care.

Relapsed MCL:

For patients who had an early relapse (before 1-2 years), targeted approaches should be considered. The current recommendation is CHOP chemotherapy followed by bendamustine or R-BAC. Some of the most common targeted therapies include bortezomib, temsirolimus, lenalidomide, and ibrutinib. Ibrutinib has been shown to achieve the best outcomes, with complete response rates (CR; no detectable cancer after treatment) between 21% and 44%. Lenalidomide is recommended for patients who cannot take ibrutinib, and should be taken with rituximab if possible (CR rates of 8%-36%). Rituximab maintenance therapy may also be effective, but more research is needed.

For elderly patients, rituximab plus radioimmunotherapy (RIT) has been shown to make remission longer. For younger, eligible patients, allogenic stem cell transplantation (allo-SCT; using stem cells from a matched donor) is a good option. It is potentially curative and has long-term remissions.

Follow-up:

There are no evidence-based recommendations for follow-up care. All recommendations are based on expert agreement. Recommendations include a check-up and blood test every 3 months for 2 years, every 6 months for 3 years, then one a year after that. PET (positron emission tomography) and CT (computed tomography) scans should not be part of routine follow-up unless relapse is strongly suspected. 

This study reviewed the ESMO guidelines for the treatment of patients with mantle cell lymphoma. 

All treatment regimens are targeted to each individual patient based on many factors including age, disease status, and other diseases that the patient may have. This article represents the general guidelines, but each individual should work with their doctor to create a regimen that works best and is safest for their unique situation. 

It is important to speak to your doctor about which regimen best fits your individual situation.