MEDizzy - A patient with primary Sjögren’s syndrome that was diagnosed

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A patient with primary Sjögren’s syndrome that was diagnosed 6 years ago and treated with tear replacement for symptomatic relief notes continued parotid swelling for the last 3 months. She has also noted enlarging posterior cervical lymph nodes. Evaluation shows leukopenia and low C4 complement levels. What is the most likely diagnosis?

ExplanationLymphoma is well known to develop specifically in the late stage of Sjögren’s syndrome. Common manifestations of this malignant condition include persistent parotid gland enlargement, purpura, leukopenia, cryoglobulinemia, and low C4 complement levels. Most of the lymphomas are extranodal, marginal zone B cell, and low grade. Low-grade lymphomas may be detected incidentally during a labial biopsy. Mortality is higher in patients with concurrent B symptoms (fevers, night sweats, and weight loss), a lymph node mass greater than 7 cm, and a high or intermedi - ate histologic grade