Peter Martin, MD, discusses the differences between subgroups of mantle cell lymphoma. MCL is a heterogenous disease, and in the World Health Organization classification of lymphoid neoplasms, MCL has a leukemia non-nodal presentation, says Martin.
Peter Martin, MD, assistant professor of medicine, Division of Hematology/Oncology, Weil Cornell Medicine, discusses the differences between subgroups of mantle cell lymphoma (MCL). MCL is a heterogenous disease, and in the World Health Organization classification of lymphoid neoplasms, MCL has a leukemia non-nodal presentation, says Martin.
MCL cells that evolve from a cell that passes through the germinal center tend to beSOX11-negative,IGHV-mutant, and behave more indolently, Martin says. These patients may also go on to develop aTP53mutation and blastoid transformation. The MCL cells that have not passed through the germinal center, on the other hand, represent more classic MCL subtypes. These patients tend to beSOX11-positive andIGHV-unmutated, and these patients have a worse prognosis, Martin says, compared with the non-nodal patients. However, about 20% of these patients can be observed for a significant period of time.
Other groups of patients with MCL that tend to do worse include the blastoid morphology that comes with a more proliferative state and those withTP53mutations or high p53 protein expression. Martin says that oftentimes, Ki-67, blastoid morphology, and p53 go hand-in-hand, but this is not universally true. These patients should be treated differently; patients with p53 mutations who are not blastoid, for example, can be managed with chemotherapy-free approaches. Patients with blastoid morphology and p53 mutations, however, should be offered allogenic stem cell transplant early on or be considered for a clinical trial with CAR T cells, Martin concludes.