Management of Marginal Zone Lymphoma: A Canadian Perspective

Abstract

Marginal zone lymphomas (MZL) are a rare, heterogenous group of lymphomas, accounting for 5-17% of indolent non-Hodgkin lymphomas in the western world. They can be further divided into three subtypes: extranodal MZL, splenic MZL, and nodal MZL. These subtypes differ in clinical presentation and behavior, which influences how they are managed. There is currently no standard of care for the treatment of MZL, owing to the difficulty in conducting phase 3 randomized trials in MZL, and the fact that there are limited data on the efficacy of therapy in individual subtypes. Treatment practices are thus largely borrowed from other indolent lymphomas and are based on patient and disease characteristics, as well as access to therapy. This review summarizes the Canadian treatment landscape for MZL and how these therapies may be sequenced in practice.

Keywords: Bruton’s tyrosine kinase inhibitor; anti-CD20 monoclonal antibody; chemoimmunotherapy; marginal zone lymphoma; mucosa-associated lymphoid tissue lymphoma.

Figure 1

Suggested algorithm for the treatment of marginal zone lymphoma in Canada. ^a If patient is positive for Hepatitis C infection, treat first with antiviral therapy if immediate disease control is not needed. ^b Bulky disease is not clearly defined for MZL and may be considered as >7 cm as per GELF (Groupe d’Etude des Lymphomes Folliculaires) criteria for follicular lymphoma [104] or >10 cm, depending on individual practice. ^c H. pylori eradication therapy should be confirmed ≥4 weeks after treatment. If initial eradication therapy is unsuccessful, up to two additional trials of H. pylori eradication can be considered. ^d If H. Pylori eradication is confirmed, then serial scopes with biopsies should be performed every 3 months until a complete lymphoma response is reached. ^e Testing for C. psittaci in ocular adnexal MALT lymphoma is not routinely performed in Canada but may be considered. In patients who are C. psittaci-positive and do not require immediate disease control treatment with doxycycline may be considered. ^f Stage I NMZL is extremely rare, but ISRT may be considered in these patients based on extrapolation from studies in follicular lymphoma suggesting a chance for a functional cure [105,106]. ANC, absolute neutrophil count; BR, bendamustine-rituximab; Bruton’s tyrosine kinase; BSC, best supportive care; CHOP-R, cyclophosphamide-doxorubicin-vincristine-prednisone-rituximab; Clb-R, chlorambucil-rituximab; CVP-R, cyclophosphamide-vincristine-prednisone-rituximab; EMZL, extranodal marginal zone lymphoma; FR, fludarabine-rituximab; hgb, hemoglobin; ISRT, involved-site radiation therapy; MALT, mucosa-associated lymphoid tissue; NMZL, nodal marginal zone lymphoma; plt, platelets; R2, rituximab-lenalidomide; SAR, single-agent rituximab; SMZL, splenic marginal zone lymphoma.