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Pathology · Autoimmunity: Sjogren syndrome
Sample itemA 52-year-old woman with dry eyes, dry mouth and anti-SSA (Ro) antibodies develops persistent parotid enlargement, palpable purpura and low C4. Which malignancy is she at increased risk of?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. Marginal zone (MALT-type) B-cell lymphoma
Sjogren syndrome involves lymphocytic destruction of lacrimal and salivary glands. Chronic B-cell stimulation predisposes to marginal zone lymphoma, often in the parotid, and the risk is highest with persistent parotid swelling, palpable purpura, cryoglobulinaemia and low C4. New enlargement of a gland in a Sjogren patient needs biopsy.
Option by option
A. Marginal zone (MALT-type) B-cell lymphoma (correct)
Correct. Marginal zone lymphoma is the classic lymphoma complication of Sjogren syndrome.
B. Classical Hodgkin lymphoma
Hodgkin lymphoma is not the characteristic lymphoma in Sjogren syndrome.
C. T-lymphoblastic lymphoma
T-lymphoblastic lymphoma is a tumour of children and adolescents, with a mediastinal mass.
D. Burkitt lymphoma
Burkitt lymphoma is linked to MYC translocation and EBV in endemic disease.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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