Mantle cell lymphoma medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]

Overview

The predominant therapy for mantle cell lymphoma is chemotherapy. Adjunctive immune based therapy, radioimmunotherapy, and new biologic agents may be required.

Medical Therapy

There are no proven standards of treatment for mantle cell lymphoma, and not even consensus among specialists on how to treat it optimally. Many regimens are available and often get good response rates, but patients almost always get disease progression after chemotherapy. Each relapse is typically more difficult to treat, and relapse is generally faster. Fortunately, regimens are available that will treat relapse, and new approaches are under test. Because of the aforementioned factors, many MCL patients enroll in clinical trials to get the latest treatments.

• There are four classes of treatments currently in general use:

• Chemotherapy
• Immune based therapy
• Radioimmunotherapy
• New biologic agents

• The phases of treatment are generally:

• Frontline
• Following diagnosis
• Consolidation
• After frontline response (to prolong remissions)
• Relapse (Relapse is usually experienced multiple times.)

Chemotherapy

Chemotherapy is widely used as frontline treatment, and often is not repeated in relapse due to side effects. Alternate chemotherapy is sometimes used at first relapse.

Frontline treatment

• Drug Regimen: CHOP (IV) (Cyclophosphamide, AND Doxorubicin, AND Vincristine, AND Prednisone) PLUS Rituximab
• Drug Regimen: Fludarabine
• Drug Regimen: Fludarabine ± (Cyclophosphamide AND Mitoxantrone AND Rituximab)

Elderly(over 65) patients, baseline beta-2 microglobulin blood test was normal

• Drug Regimen: Hyper-(CVAD) Course A:Cyclophosphamide, AND Vincristine, AND Doxorubicin, AND Dexamethasone ; Course B : Methotrexate AND Cytarabine

Relapsed patients

• Drug regimen: PEP-C (oral), Prednisone, AND Etoposide, AND Procarbazine, AND Cyclophosphamide[3].
• Chemotherapy ± Total body irradiation (TBI)

Immunotherapy

Immune-based therapy is dominated now by the oft used and effective rituximab monoclonal antibody. It can have good activity against mantle cell lymphoma alone but especially in combination with chemotherapies to prolong response duration. Rituximab essentially tags the cancer cells for destruction by the body. There are newer variations on monoclonal antibodies combined with radioactive molecules known as Radioimmunotherapy (RIT). These include Zevalin and Bexxar.

Targeted Therapy

New targeted agents include the proteasome inhibitor Velcade and mTor (mammalian target of rapamycin) inhibitors such as temsirolimus.