What is Stage IV Mantle Cell Lymphoma?

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Mantle cell lymphoma (MCL) is a relatively rare B-cell subtype of non-Hodgkin's lymphoma (NHL). About 6 percent of NHL's diagnosed in the United States each year are mantle cell lymphomas. They are called this because the cancer begins in the mantle zone of the lymphocyte.

You can learn more about the mantle and how this subtype got its name by reading What is the Mantle?

Understanding MCL

Doctors' understanding of mantle cell lymphoma is in constant flux because the discovery of the subtype was relatively recent. Up until a few years ago, it was believed that MCL was a slow-growing cancer that presented little threat to the life of the patient.

That notion has been turned upside-down: It is now believed that four of every five patients with MCL will require aggressive treatment in the form of combination chemotherapy and a stem cell transplantation. Meanwhile, one of five patients will have an indolent disease and require little intervention. The difficulty is in determining who has which.

Stage IV Disease

MCL has a rather poor prognosis, owning largely to the fact that in the majority of cases, the disease isn't discovered until it has become advanced — meaning it has spread from the primary site to lymph nodes on both sides of the diaphragm and has also spread into the gastrointestinal tract as well as bone marrow. When it has spread to nodal groups both above and below the diaphragm and involves an organ or organ system, it is considered stage IV disease.

Treating Advanced, Stage IV Mantle Cell Lymphoma

There is currently no clear-cut consensus on the best specific front-line therapy for stage IV MCL. However, the general consensus is one of several forms of combination chemotherapy followed by immunotherapy maintenance, a stem cell transplant or, in some cases, recommendation of a clinical trial.

The good news is that ongoing clinical trials are bringing treatment options into better focus and establishing some potential therapies for future use. One of them involves combination chemotherapy followed by a therapeutic vaccine as consolidation therapy. In a recent interview, one of the nation's foremost experts on MCL, Dr. Eduardo Sotomayor, discussed the success he and his colleagues are seeing with this approach.

With the creation of groups such as the Mantle Cell Lymphoma Consortium, it is a little easier to imagine a future in which Stage IV mantle cell lymphoma is a manageable and treatable disease.

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