Splenic marginal zone lymphoma (SMZL) presenting alongside active chronic hepatitis C virus (HCV) infection defines a distinct clinical subgroup. The presence of HCV co-infection directly shapes the sequence and selection of management strategies.
This protocol addresses patients with SMZL who have concomitant chronic HCV infection. In those who do not immediately require conventional lymphoma-directed therapy, antiviral treatment targeted at HCV should be evaluated as part of the overall management plan, given the recognised association between HCV and this lymphoma subtype.
Initial lymphoma-directed therapy centres on a targeted antibody-based approach; the full protocol — including sequencing, eligibility thresholds, maintenance strategy, and clinical decision criteria — is available in the structured regimen.
In patients with concomitant chronic HCV infection who do not immediately need conventional treatment, antiviral therapy should be considered [IV, B].
Anti-HCV therapy should be considered in patients with HCV-associated lymphoma [IV, B].
Rituximab therapy alone (375 mg/m2 4- to 8-weekly doses) can produce a rapid response, with a high overall response rate (>80%) and CRR (>40%) with minimal toxicity.
Maintenance with rituximab (every 2 months for 1-2 years) may improve PFS, but no OS advantage has been shown to date and maintenance may not be superior to retreatment on demand.
Rituximab alone is the preferred initial therapy in patients with SMZL [III, A].
Achievement of CR is defined by normal spleen size, normal blood counts, negative flow cytometry on blood and negative IHC on bone marrow biopsy.
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