Which malignancy has been associated with Sjogren's syndrome?

• A. CLL
• B. CML
• C. Non-Hodgkin lymphoma
• D. Hodgkin lymphoma

Answer: (C)

Sjögren's syndrome increases the risk of B-cell lymphoproliferative disorders, especially a type of non-Hodgkin lymphoma. The autoimmune drive in Sjögren's causes chronic stimulation and expansion of B cells, particularly in the exocrine glands. This environment can lead to clonal B-cell growth and malignant transformation, so patients may develop non-Hodgkin lymphoma, often in the salivary glands or other extranodal sites. Features that raise suspicion include persistent gland enlargement, new or enlarging lymphadenopathy, cryoglobulinemia, low complement levels (especially C4), and sustained autoantibody activity (like rheumatoid factor).

Mechanistically, ongoing antigen stimulation keeps B cells activated, and factors like BAFF (a B-cell activating factor) promote survival of autoreactive B cells, increasing their chance to accumulate mutations and become malignant. While Hodgkin lymphoma is a hematologic malignancy, Sjögren's has a stronger, more established association with non-Hodgkin B-cell lymphomas, making that malignancy the best match in this context.