Mantle cell lymphoma (MCL) is a rare, biologically and clinically heterogeneous mature B cell lymphoma, representing 5% to 10% of non-Hodgkin lymphomas (NHLs) in the United States.1,2
There are 4 morphological variants: small-cell, marginal zone–like, pleomorphic, and blastoid; patients with marginal zone-like variant MCL frequently exhibit pronounced splenomegaly.
MCL is more than twice as common among men and tends to be diagnosed in late adulthood, with a median age at diagnosis of 68 years.2-4 Rates are highest among non-Hispanic Caucasian males.
Optimal treatment is “highly individualized,” according to Peter Martin, MD, of the division of hematology and medical oncology at Weill Cornell Medical College in New York, New York, and coauthors of a recent review of the standards of care for MCL.1
“The treating physician must consider patient and disease-related factors, balancing the goals of therapy with expected therapy-related toxicities,” they reported. “[I]t demands constant attention; clinicians must continually integrate new data while researchers are constantly pushed to develop new options. Fortunately, these efforts appear to be paying off with significant improvements in overall survival over the past decade.”
Despite those advances, prognosis remains poor and the goal of treatment is rarely curative, though risk stratification is used to identify patients with low-risk and aggressive disease.
Deferred treatment is sometimes an option for newly diagnosed, asymptomatic patients with low-risk disease. Approximately 1 in 4 patients with MCL has indolent disease at diagnosis, for which active surveillance is the preferred approach.
Criteria for identifying these patients include non-blastoid-variant morphology, low tumor burden, normal LDH, and Ki67 less than 30%, as well as an absence of “B” symptoms (fever, night sweats, and weight loss).1
“Additionally, some patients with chronic lymphocytic leukemia (CLL)-like presentation with leukemic disease in the absence of adenopathy, mutated IGVH, and lack of SOX11 expression appear to have a more indolent initial clinical course,” Dr Martin’s team noted.
The Mantle Cell International Prognostic Index (MIPI) does not, however, reliably identify patients with indolent disease.
“We…appear to be entering an era when emerging technologies and novel agents might be combined in pilot studies earlier in the course of the disease with the goal of potentially prolonging the time to more intensive therapies,” the authors reported.
Jose D. Sandoval-Sus, MD, of the H. Lee Moffitt Cancer Center and Research Institute in Tampa, Florida, and colleagues describe an active surveillance schedule for asymptomatic patients with low-risk disease involving complete history, physical exam, complete blood count, complete metabolic panel, and LDH every 3 to 6 months.2
Once a patient becomes symptomatic, develops cytopenias, or develops symptomatic, rapidly-growing lymphadenopathy, or splenomegaly, systemic treatment is initiated.