BACKGROUND: Mantle cell lymphoma (MCL) remains incurable using conventional chemotherapeutic approaches. New clinical data show that some patients have a chronic/indolent course and others have a more fulminant course and short survival, similar to that of patients with acute leukemias. METHODS: This review presents an overview of this aggressive disease, including the diagnosis, epidemiology, prognosis, and management of this protean and challenging condition. RESULTS: Distinguishing indolent MCL from in situ MCL is important but can be challenging. Molecular exploration has identified SOX11 and HDAC11 as potential candidate genes for discrimination of indolent cases. Improvements in the prognosis in MCL are likely the result of earlier identification of more indolent cases and the application of modern modalities, including rituximab and autologous transplantation. Younger patients may be able to tolerate more intensive therapy, while treatment for elderly or frail patients may focus on maintenance to prolong remission. For patients with relapsed disease, some agents have shown promise, such as lenalidomide and bortezomib. Emerging drugs such as PCI37625 and CAL-101 are being explored in phase I and II studies. CONCLUSIONS: Although patients with MCL continue to experience poor outcomes, new treatment approaches for various stages of disease are showing promise in improving survival.
BACKGROUND:Mantle cell lymphoma (MCL) remains incurable using conventional chemotherapeutic approaches. New clinical data show that some patients have a chronic/indolent course and others have a more fulminant course and short survival, similar to that of patients with acute leukemias. METHODS: This review presents an overview of this aggressive disease, including the diagnosis, epidemiology, prognosis, and management of this protean and challenging condition. RESULTS: Distinguishing indolent MCL from in situ MCL is important but can be challenging. Molecular exploration has identified SOX11 and HDAC11 as potential candidate genes for discrimination of indolent cases. Improvements in the prognosis in MCL are likely the result of earlier identification of more indolent cases and the application of modern modalities, including rituximab and autologous transplantation. Younger patients may be able to tolerate more intensive therapy, while treatment for elderly or frail patients may focus on maintenance to prolong remission. For patients with relapsed disease, some agents have shown promise, such as lenalidomide and bortezomib. Emerging drugs such as PCI37625 and CAL-101 are being explored in phase I and II studies. CONCLUSIONS: Although patients with MCL continue to experience poor outcomes, new treatment approaches for various stages of disease are showing promise in improving survival.
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