Peter Martin, MD, chief, the Lymphoma Program, Meyer Cancer Center, associate professor of medicine, Weill Cornell Medicine, discusses the heterogeneity of mantle cell lymphoma (MCL).
Researchers are beginning to better appreciate the heterogeneity of MCL, says Martin. Biologically, MCL has a leukemic non-nodal presentation and is categorized as such in the World Health Organization classification of lymphoid neoplasms. MCL cells tend to develop from a cell that passes through the germinal center reaction; they tend to be SOX11-negative, IGHV-mutated, and more indolent. Some of these patients may go on to develop TP53 mutations and blastoid transformation.
Additionally, there are more classical presentations of the disease. These typical nodal patients have MCL cells that have not passed through the germinal center; as such, they are mainly SOX11-positive and IGHV unmutated. Typically, these patients have a worse prognosis than the leukemic non-nodal patients, says Martin. However, approximately 20% can receive active surveillance for a span of several years. There are data to suggest that many of these patients can do well for a couple of years without therapy, he adds.
There is also the blastoid morphology variant that has a more proliferative state. These patients with TP53 mutations or high p53 protein expression tend to have a worse prognosis, says Martin. Oftentimes, Ki-67, blastoid morphology, and p53 go hand-in-hand, but they can be found separately as well. Patients with p53 mutations who do have not blastoid morphology can be managed with non-chemotherapy approaches, whereas patients with blastoid morphology and p53 mutations should be offered a more aggressive approach with allogenic stem cell transplant or a clinical trial with CAR T cells, concludes Martin.