Recent advances in treatment of nodal and gastrointestinal follicular lymphoma

Table 6 Summary of clinical trial results of epigenetic regulators

Study	Targeted disease	No. of patients	Objective response rate	Complete response rate	Progression-free survival	Overall survival	Adverse events or others	Ref.
Tazemetostat alone, phase-II	r/r FL, EZH2-mut; FL, EZH2-wt. FL	n = 99, mut FL n = 45; wt FL n = 54	69% (EZH2 mut); 35% (EZH2 wt)	Unknown; unknown	Median PFS: 13.8 mo (EZH2 mut); 13.1 mo (EZH2 wt)	Unknown	G3 or higher 27%+, treatment discontinued at 8%	[54]
Tazemetostat (first EZH2 inhibitor) vs inderalisib, duvelisib, copanlishib, umbralisib	r/r FL, systematic literature review		Tazemetostat vs inderalisib 43% vs 56; duvelisib 48% vs 47; Kopanlisib 49% vs 61; umbralisib 57% vs 47; no significant difference in either case	Unknown	Unknown	Unknown	Predominantly reduced risk of adverse events compared to PI3Ki	[57]
Vorinostat (HDACi), phase-II	r/r Inhl + MCL, median with one or more prior treatment	n = 39 (r/r FL)	49%	Unknown	Median PFS, 20 mo	Unknown	G3 or higher 8%	[58]
Vorinostat + rituximab, phase-II	Untreated and r/r FL (4 or less prior treatment)	n = 22	46% (all patients); 67% (untreated pts); 41% (r/r FL)	Unknown	Median PFS, 29.2 mo (all patients); not reached (untreated pts); 18.8 mo (r/r FL)	Unknown		[59]
Mocetinostat, phase-II	r/r DLBCL, r/r FL	n = 41, n = 31	18.9% (r/r DLBCL), 11.5% (r/r FL)	Unknown	1.8-22.8 mo (DLBCL); 11.8-26.3 mo (FL)	Unknown	Fatigue (75.0%); nausea (69.4%); diarrhea (61.1%)	[60]

“Unknown” means data not shown, unknown information or not reached. r/r: Refractory and relapsed; FL: Follicular lymphoma; EZH2: Enhancer of zeste homolog 2; mt: Mutant; wt: Wild type; (i)NHL: (Indolent) non-Hodgkin lymphoma; DLBCL: Diffuse large B-cell lymphoma; MZL: Marginal zone lymphoma; MCL: Mantle cell lymphoma; G: Grade; PI3Ki: Phosphoinositide 3 kinase inhibitor.