Progress in molecular feature of smoldering mantle cell lymphoma

Abstract

Mantle cell lymphoma (MCL) is considered one of the most aggressive lymphoid tumors. However, it sometimes displays indolent behavior in patients and might not necessitate treatment at diagnosis; this has been described as “smoldering MCL” (SMCL). There are significant differences in the diagnosis, prognosis, molecular mechanisms and treatments of indolent MCL and classical MCL. In this review, we discuss the progress in understanding the molecular mechanism of indolent MCL to provide insights into the genomic nature of this entity. Reported findings of molecular features of indolent MCL include a low Ki-67 index, CD200 positivity, a low frequency of mutations in TP53, a lack of SOX11, normal arrangement and expression of MYC, IGHV mutations, differences from classical MCL by L-MCL16 assays and MCL35 assays, an unmutated P16 status, few defects in ATM, no NOTCH1/2 mutation, Amp 11q gene mutation, no chr9 deletion, microRNA upregulation/downregulation, and low expression of several genes that have been valued in recent years (SPEN, SMARCA4, RANBP2, KMT2C, NSD2, CARD11, FBXW7, BIRC3, KMT2D, CELSR3, TRAF2, MAP3K14, HNRNPH1, Del 9p and/or Del 9q, SP140 and PCDH10). Based on the above molecular characteristics, we may distinguish indolent MCL from classical MCL. If so, indolent MCL will not be overtreated, whereas the treatment of classical MCL will not be delayed.

Exploration of mRNAs and miRNA classifiers for various ATLL cancer subtypes using machine learning

“…Furthermore, the expression of PARP1 is related to a progressive course of indolent mantle cell lymphoma. Therefore, it was proposed that PARP1 could be used for the initial diagnostic studies as a negative predictor [42].…”

The implication of next-generation sequencing in the diagnosis and clinical management of non-Hodgkin lymphomas

“…Indolent leukemic non-nodal MCL is characterized by mutated IGHV and primarily SOX11 negativity, as well as peripheral blood, bone marrow, and occasionally splenic involvement but no major nodal involvement. Classical MCL is characterised by unmutated or minimally mutated IGHV and mostly SOX11 positivity (6,58).…”

Tackling Mantle Cell Lymphoma in Europe

“…Molecular-based agents together with chemoimmunotherapy may be appropriate for some patients but potential toxicity needs to be taken into consideration. Treatments differ with regards to efficacy, adverse event profiles and mode of administration, and the choice of treatment is also dependent on factors such as patient fitness and wishes [ 5 , 10 , 21 ]. The Mantle Cell Lymphoma International Prognostic Index (MIPI), the first prognostic index for MCL patients, is based on independent prognostic factors including age, Eastern Cooperative Oncology Group (ECOG) performance status, lactate dehydrogenase (LDH) level, and leucocyte count.…”