Mantle cell lymphoma (MCL), one of the B cell non-Hodgkin lymphomas (NHL), has a variable course. A minority of patients with this disorder may survive untreated for many years. However, more frequently, MCL assumes a more virulent character, akin to that of an aggressive NHL variant.
There is great variability in the initial treatment of MCL used in clinical practice. While some advocate intensive chemotherapy with autologous transplantation, others prefer combination chemotherapy regimens used for aggressive NHL subtypes. (See "Initial treatment of mantle cell lymphoma".)
Despite all efforts to the contrary, therapy for MCL is not curative and virtually all patients will have refractory or recurrent disease. Treatment of recurrent MCL is difficult, due to the rapid development of chemotherapy resistance. There are multiple chemotherapy regimens that may be tried and clinical practice varies greatly by center. Given the limited efficacy of these agents and paucity of data comparing these various treatment options, participation in a clinical trial is encouraged whenever possible.
The treatment of relapsed or refractory mantle cell lymphoma will be discussed here. The initial treatment of mantle cell lymphoma and the clinical and pathologic features of this disorder are discussed separately. (See "Initial treatment of mantle cell lymphoma" and "Clinical manifestations, pathologic features, and diagnosis of mantle cell lymphoma".)
Overview — A number of chemotherapy regimens have been evaluated in patients with recurrent or refractory MCL, with variable success. Some of these agents are undergoing further investigation for use in combination therapies. All patients should be encouraged to participate in clinical trials whenever available.