American Board of Internal Medicine (ABIM) Certification Practice Exam

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What type of lymphoma do patients with Sjögren syndrome face an increased risk of developing?

T-cell lymphoma
Diffuse B-cell lymphoma
Follicular lymphoma
Burkitt lymphoma

The correct answer is: Diffuse B-cell lymphoma

Patients with Sjögren syndrome have an increased risk of developing diffuse large B-cell lymphoma, which is a type of non-Hodgkin lymphoma. This association is mainly due to the chronic immune stimulation and inflammation seen in Sjögren syndrome, which can lead to the malignant transformation of lymphocytes. The features of Sjögren syndrome, such as lymphocytic infiltration of exocrine glands and the presence of autoantibodies, are believed to create a microenvironment that predisposes individuals to the development of B-cell lymphomas, particularly the aggressive subtype of diffuse large B-cell lymphoma. Other types of lymphoma listed, while they may occur in the general population, do not have the same established correlation with Sjögren syndrome as diffuse large B-cell lymphoma does. Therefore, the specific relationship between Sjögren syndrome and an increased incidence of diffuse large B-cell lymphoma underscores the importance of monitoring for hematological complications in patients with this autoimmune condition.