Should There Be a Standard Therapy for Mantle Cell Lymphoma?

Mitchell R Smith

Disclosures

Future Oncol. 2011;7(2):227-237. 

In This Article

Abstract and Introduction

Abstract

Mantle cell lymphoma (MCL) is an uncommon subtype of B-cell lymphomathat is characterized by monoclonal B cells that express CD5 on their surface, but not CD23, and harbor the t(11:14) chromosomal translocation that leads to dysregulated expression of cyclin D1. MCL is a biologically and clinically heterogeneous disease. It has the unfavorable characteristics of both aggressive and indolent lymphoma in that MCL is not curable with current standard therapy, yet patients have a shorter survival compared with other indolent histology. MCL is incurable, yet more intensive therapy does lead to longer disease-free intervals; therefore, treatment must be designed to optimize survival while maintaining quality of life. Thus, therapy should be individualized based on both the clinical behavior of the lymphoma and the patient's status. While there is no clear standard therapy that can be recommended for all patients, there may be an optimal choice for each patient. Knowledge of the expected clinical benefits and toxicities of various approaches will allow the physician and patient to appropriately select the therapy.

Introduction

Patients with mantle cell lymphoma (MCL) are quite heterogeneous in terms of their clinical characteristics as well as the underlying disease process itself. Clearly, the answer to the question posed in the title of this article is that there is not a single optimal therapy. The question is whether we can define an optimal therapy for an individual patient based upon clinical and biologic features of that patient's presentation. During the last decade, we have made significant strides towards defining subsets of patients who require and tolerate more intensive regimens and likely benefit from these interventions, even though they are not curative, and, at the other end of the spectrum, patients who either do not require such intense therapy and/or cannot tolerate these regimens and are better served by alternative approaches. Clinical determinates are primarily age, performance status and comorbidities. The primary biologic characteristic is lymphoma proliferative rate, which is assayed by various parameters such as Ki-67 or microarray gene expression signatures. The clinical question is whether we can tailor our selected treatment regimen based on clinicopathologic features in order to optimize benefit and minimize unnecessary risk and toxicity.

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