This protocol applies to patients with splenic marginal zone lymphoma (SMZL) who do not have hepatitis C infection, who had a clinical indication for treatment, and whose disease relapsed or did not achieve an adequate response after first-line systemic therapy.
The patient has SMZL without hepatitis C infection. An indication for treatment was present — including B symptoms, progressive cytopenia, symptomatic splenomegaly, bulky lymphadenopathy, autoimmune phenomena, or disease compromising vital organs. First-line therapy was initiated accordingly.
Initial management — which may have included rituximab monotherapy, immunochemotherapy combinations, or splenectomy — did not achieve a complete response. Complete response required:
Failure to reach these endpoints defines relapsed or refractory SMZL and escalates management to this next-line protocol.
Several categories of agents are available for relapsed or refractory SMZL — including targeted therapies acting on key B-cell signalling pathways and immunotherapy-based combinations. The full options, selection criteria, and sequencing are detailed in the structured protocol.
DOI: 10.1111/bjh.19064
The main criteria for treatment include constitutional symptoms, symptomatic or progressive splenomegaly, bulky lymphadenopathy, autoimmune phenomena and progressive cytopenias, as shown in Table 9.
SMZL patients with hepatitis C infection should receive up-front anti-viral therapy.
There is limited evidence that rituximab monotherapy can be effective for relapsed/refractory SMZL, including those who have progressed after rituximab monotherapy or splenectomy.
Treatment for relapsed MZL follows a similar paradigm to relapsed/refractory follicular lymphoma, including RT for localised relapse and active surveillance for asymptomatic, advanced stage relapse.
Bruton tyrosine kinase (BTK) plays a critical role in MZL pathogenesis, and several covalent BTK inhibitors (cBTKi) are approved for relapsed/refractory MZL.
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