Sjögren Syndrome
ICD-10 M35.0 ICD-11 4A43.2

Sjögren Syndrome with Diffuse Large B-Cell Lymphoma

Patients with primary Sjögren syndrome carry an elevated risk of B-cell lymphoma development. When that lymphoma is diffuse large B-cell lymphoma (DLBCL) — a moderate-to-high-grade haematological neoplasia — the clinical picture calls for a distinct management strategy.

This protocol addresses the treatment of Sjögren syndrome in the specific setting of concurrent diffuse large B-cell lymphoma.

Clinical Scenario

Three subtypes of B-cell lymphoma account for more than 90% of reported cases in primary Sjögren syndrome: MALT lymphoma, other marginal zone lymphomas, and diffuse large B-cell lymphoma. DLBCL represents a moderate-to-high-grade category within this spectrum and places the patient in a distinct therapeutic group that differs from lower-grade subtypes.

Treatment Approach (partial)

For Sjögren syndrome patients with diffuse large B-cell lymphoma, the haematological grade of the neoplasia is central to treatment selection. Management involves a rituximab-based chemotherapy regimen — the specific regimen, sequencing, and full treatment details are contained in the complete protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1136/annrheumdis-2019-216114

  1. 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.
  2. For patients with moderate/high grade haematological neoplasia, treatment is based on standard rituximab-based chemotherapy regimens.
  3. The benefit of adding rituximab to chemotherapy has been demonstrated in a meta-analysis in patients with follicular lymphomas, mantle cell lymphomas and other indolent lymphomas.
View source ↗