Table 5. Treatment strategies for newly-diagnosed marginal zone lymphoma.

Stage	Stratification 1	Stratification 2	Category I recommendations	Category II recommendations
HCV, hepatitis C virus; R-CHOP, R-CHOP, rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone; R-CVP, rituximab, cyclophosphamide, vincristine and prednisone.
I−II	Extra-nodal	Gastric primary	Helicobacter pylori eradication therapyRadiotherapy (Level 2A)
		Non-gastric primary	Radiotherapy (Level 2A)	Rituximab (Level 2A)
	Nodal		Radiotherapy (Level 2A)	Rituximab (Level 2A)
	Spleen	HCV positive	Anti-HCV therapy (Level 2A)
	Maintenance	HCV negative	Rituximab (Level 2A) Splenectomy (Level 2A)
III−IV	Asymptomatic		Observation (Level 2A)	Clinical trial (Level 2A)
	Symptomatic	Front-line	Rituximab + chlorambucil (Level 1B) Rituximab + bendamustine (Level 2A) R-CHOP (Level 2A) R-CVP (Level 2A) Rituximab + lenalidomide (Level 2A)	Clinical trial (Level 2A) Rituximab + chemotherapy followed by rituximab maintenance (Level 2A) Rituximab + fludarabine (Level 2A)
		Second-line	Rituximab + bendamustine (Level 2A) R-CHOP (Level 2A) R-CVP (Level 2A) Rituximab + lenalidomide (Level 2A)	Ibrutinib (Level 2A) Zanubrutinib (Level 2A)