Mantle Cell Lymphoma

Last Modified: September 28, 2006

Dear OncoLink "Ask The Experts,"

My uncle has just been diagnosed with mantle cell lymphoma and we are not sure of the outcomes with this type of cancer. We would like more information on this disease.

Babis (Charalambos) Andreadis, MD, Assistant Professor in Medicine in the Division of Hematology/Oncology at the Abramson Cancer Center and an Associate Scholar in the Center for Clinical Epidemiology and Biostatistics at the University of Pennsylvania, responds:

Mantle cell lymphoma (MCL) is a subtype of non-Hodgkin's lymphoma (NHL) that has been recognized over the past decade or so. It is a relatively rare subtype of NHL that usually occurs in the 5th or 6th decade of life. It can present with swollen lymph glands or with symptoms of the gastrointestinal tract, such as nausea, heartburn, diarrhea, and weight loss. Rarely, lymphoma cells can be found circulating in the bloodstream. Once the diagnosis is made, patients with mantle cell lymphoma have extremely variable clinical outcomes, some with very slow-growing disease, and some with a rapidly progressive course. Unfortunately, there is no proven cure for this disease, with the average patient succumbing to lymphoma in about 3 years.

As of today, there is no widely accepted standard of care for mantle cell lymphoma. The goals of treatment are therefore tailored to each individual patient, taking into account the patient's age, other medical conditions, the rate of growth of the disease, and the patient's desire for aggressiveness in treatment. Someone who is older, has other medical problems, and with slow-growing disease may elect to receive no treatment upfront and wait until the disease progresses (the "watch and wait" approach). On the other end of the spectrum, a young patient in good health with rapidly progressing symptomatic disease should initiate therapy as soon as possible.

Once the decision to start therapy is made, there is a variety of chemotherapy regimens from which to choose. Active regimens with a proven clinical record include R-CHOP, R-CVP, R-FCM, a regimen called R-hyperCVAD, and others. These generally produce a response rate of upwards of 80% of patients, with a complete disappearance of detectable disease in 40% to 70% of patients, depending on the regimen. However, even a complete response does not bring about a cure, as the majority of patients will eventually experience a relapse within 1 to 2 years of treatment. Given these outcomes, several groups nationally and internationally are looking for ways to prolong the first response to chemotherapy. For selected patients who are young and desire to be very aggressive, high-dose chemotherapy followed by a stem cell transplant can prolong the duration of this response as well as patient survival, to upwards of 3 years. Finally, several novel agents are active in this disease, including thalidomide (Thalomid) and bortezomib ( Velcade) , and several others are being studied in clinical trials. Because it is unclear which one of these approaches will emerge as the standard of care for mantle cell lymphoma, enrollment in clinical trials should be considered for all patients.