Treatment of Sjögren Syndrome with Low-Grade B-Cell Lymphoma (MALT, Marginal Zone, SLL, or LPL)

Primary Sjögren syndrome carries a substantially elevated risk of lymphoma. When low-grade B-cell lymphoma develops — including MALT lymphoma, marginal zone lymphoma (MZL), small lymphocytic lymphoma (SLL), or lymphoplasmacytic lymphoma (LPL) — the clinical situation requires a specific, histology-guided treatment approach beyond routine Sjögren syndrome management.

Clinical Scenario

Low-grade B-cell lymphoma complicating primary Sjögren syndrome. Three subtypes — MALT lymphoma, other marginal zone lymphomas, and diffuse large B-cell lymphoma — account for the great majority of lymphoma cases reported in primary Sjögren syndrome. Treatment selection must be individualised according to the specific histological subtype and disease stage.

First-Line Approach partial preview

Current evidence supports a rituximab-based combination regimen as first-line therapy for eligible patients with MZL, SLL, or LPL in the setting of primary Sjögren syndrome. The specific partner agent and full regimen details are outlined in the structured protocol below.

Instant Access to Structured Evidence-Based Regimens

References

Three subtypes of B-cell lymphoma account for more than 90% of reported cases in primary SjS: mucosa-associated lymphoid tissue (MALT) lymphoma, other marginal zone lymphomas (MZL) and Diffuse large B cell lymphoma.

Treatment of B-cell lymphoma should be individualised according to the specific histological subtype and disease stage.

Rituximab plus fludarabine or bendamustine (BR) are the recommended first-line therapy for MZL, SLL and LPL; a recent study in 13 patients with pSjS-2002 (77% stage IV) complicated by MZL has reported the efficacy of the BR combination in all 13 cases, with improvement in the other SjS non-lymphomatous manifestations and with a good safety profile.

DOI: 10.1136/annrheumdis-2019-216114

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